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LIBRARY OF CONGRESS. 

Chap. _____._. Copyright No. 

Shelf_Ai#_6 7 



UNITED STATES OF AMERICA. 



$ 



THE DISEASES OF 
THE NERVOUS SYSTEM 



A TEXT-BOOK 
FOR PHYSICIANS AND STUDENTS 



BY 

Dr. LUDWIG HIRT 

PROFESSOR AT THE UNIVERSITY OF BRESLAU 



TRANSLATED, WITH PERMISSION OF THE AUTHOR, BY 

AUGUST HOCH, M. D. 

FORMERLY ASSISTANT PHYSICIAN TO THE JOHNS HOPKINS HOSPITAL 
NOW TO THE MCLEAN HOSPITAL, WAVERLY, MASS. 

ASSISTED BY 

FRANK R. SMITH, A.M. (Cantab.), M. D. 

INSTRUCTOR IN MEDICINE IN THE JOHNS HOPKINS UNIVERSITY 

WITH AN INTRODUCTION' BY' 
WILLIAM OSLER, M. D., F. R. C. P., F. R. S. 

PROFESSOR OF MEDICINE IN THE JOHNS HOPKINS UNIVERSITY, ETC. 



WITH ONE HUNDRED AND EIGHTY-ONE ILLUSTRATIONS 



NEW YORK 
D. APPLETON AND COMPANY 

il 






30581 

Copyright, 1893, 1899, 
By D. APPLETON AND COMPANY. 

TWO COP IE. a RECEIVED. 






Electrotyped and Printed 
at the Appleton Press, U. S. A. 






1*1 



TO 

WILHELM ERB 

THIS BOOK IS DEDICATED 

AS A TOKEN OF GRATITUDE AND ESTEEM 

BY THE AUTHOR. 



ffl 



i 



rP -i ! f-r- « 



INTRODUCTION 
TO THE SECOND GERMAN EDITION. 



On the first appearance of this work some fears regard- 
ing its success were entertained not only by the author, but 
by some of his colleagues and friends. The favorable recep- 
tion accorded to the first edition has proved such fears to 
be groundless. While the interest shown by German physi- 
cians has rendered it necessary to issue a second edition within 
a comparatively short time, the translations into English, 
French, and Italian, which all came out in the same year, have 
proved that members of the profession in foreign lands have 
found much in the work that has appeared to them attractive 
and worth reading. 

The great kindness of a number of prominent American 
neurologists who have sent me reprints of their articles has 
made it possible for me to incorporate in the present edition 
several very interesting woodcuts which have been borrowed 
from these authors. I take this opportunity of expressing my 
sincere thanks to my colleagues abroad for these favors. 

The many, sometimes very essential, changes in the text, as 
well as in the arrangement of the subject-matter, will show my 
critics how readily their hints have been received. I can only 
express a hope that the work in its new form may meet with 
the same approval which it has, perhaps undeservedly, re- 
ceived on its first appearance. 

Dr. Ludwig Hirt. 

BRESLAU, February, 1894. 



INTRODUCTORY NOTE. 



The pleasure cf introducing to the profession of this country 
a translation of a standard work is enhanced by the opportu- 
nity it affords of acknowledging how great is our debt to those 
— translators and publishers — who have made current in Eng- 
lish the works of Trousseau, Niemeyer, Virchow, Cohnheim, 
and others. All recognize the necessity of teachers knowing 
the classical works in all languages, but of equal importance is 
it that the practitioners in all countries should have easy ac- 
cess by means of translations to the thoughts and experience, 
the ways and methods, of the masters of our art the world over. 
No better corrective exists to the vice of Philistinism — that nar- 
row conceit of the special prominence of medicine in any one 
country — than a wide diffusion in all of the best works of each. 

Early in 1890 my attention was called by Dr. Weir Mitchell 
to the first part of Prof. Hirt's Handbuch der Nervenkrank- 
heiten, which he characterized as an exceptionally well ar- 
ranged and thorough work on diseases of the nervous system. 
The completed work seemed in many respects so admirable a 
text-book that I wrote to Prof. Hirt and asked his permission 
to have it translated. 

The arrangement of the subjects to which the author re- 
fers in the preface, though somewhat novel, is justifiable and 
entirely satisfactory ; and it is a distinct advance in classifica- 
tion to place tabes dorsalis and dementia paralytica among 
the diseases of the general nervous system, instead of in the 
sections on diseases of the cord and diseases of the brain re- 
spectively. 

The fact which makes the work of value to the teacher, the 
student, and the practitioner is the graphic description of the 
anatomy and symptomatology of the different diseases. Where 
all is so good it is invidious to select, but the chapter on tabes 



viii INTRODUCTORY NOTE. 

is an illustration of our author's lucid and, at the same time, 
thorough treatment of his subject. The various affections are 
treated of also from an advanced modern standpoint; conflict- 
ing theories and passing observations are submitted to a wise 
criticism through which the author's own large and varied ex- 
perience is very apparent. 

An attractive aspect of the work is the excellent character 
of the illustrations, which, as they are in great part original, 
will be a pleasing relief to the hackneyed cuts which have for 
so long passed from book to book in English works. 

Pursuing the via media in the important question of treat- 
ment, neither displaying the pessimism which too many mala- 
dies of the nervous system would seem to justify, nor an opti- 
mism so flagrant as to savor of quackery, Prof. Hirt is a safe 
guide in the highways and byways of neurotherapeutics. 

And, lastly, I think the author has been fairly handled by 
his translators, who, bearing in mind the admonition of Dryden, 
" not to lackey by the side of his author, but to mount up be- 
hind him," have given a clear and interesting rendering of the 
original. 

William Osler. 

Baltimore, January, i8gj. 



CONTENTS. 



PAGE 

Diseases of the Brain and its Meninges, including those of 

the Cranial Nerves r 

PART 1. 

Diseases of the Meninges of the Brain . . ' 3 

Chap. I. — Inflammation of the inner surface of the dura mater, pachymeningitis 

interna hemorrhagica, hematoma durse matris .... 4 
II. — Inflammations of the soft membranes of the brain, leptomeningitis, puru^ 

lent meningitis 8 

PART II. 

Diseases of the Cranial Nerves 24 

Chap. I. — Diseases of the olfactory nerve 25 

II. — Diseases of the optic nerve . 29 

III. — Diseases of the nerves supplying the ocular muscles .... 42 

IV. — Diseases of the trigeminal nerve 56 

V. — Diseases of the facial nerve 77 

VI. — Diseases of the auditory nerve 95 

VII. — Diseases of the glosso-pharyngeal nerve ...... 107 

VIII. — Diseases of the vagus (pneumogastric nerve) no 

IX. — Diseases of the accessory nerve 136 

X. — Diseases of the hypoglossal nerve 140 

XL — Simultaneous affection of several cranial nerves — Multiple paralysis of 

the cranial nerves .......... 147 

PART III. 

Diseases of the Brain Proper 161 

I. The study of cerebral lesions with reference to their seat — Topical diagnosis 

— Doctrine of localization 162 

Symptoms referable to cortical lesions ....... 164 

Symptoms referable to lesions of the white matter of the hemispheres and 

to lesions of the basal ganglia 189 

II. The study of cerebral lesions with reference to their pathological nature — 

Pathological diagnosis 209 

Affections of the brain due to disease of the blood-vessels . . . 209 

A. Diseases of the cerebral vessels and their consequences . . . 209 

ix 



X CONTENTS. 

PAGE 

1. Cerebral haemorrhage 213 

2. Embolism and thrombosis of the cerebral arteries — Encephalo- 

malacia 244 

3. Endarteritis (syphilitica) 252 

4. Dilatation of the arteries of the brain 253 

5. The neuroses of the arteries of the brain (anaemia and hyperemia 

of the brain) 254 

B. Diseases of the cerebral veins and sinuses 257 

Inflammatory processes of the brain substance 260 

1. Purulent encephalitis — Brain abscess 260 

2. Nonsuppurative encephalitis and its consequences (" athetosis") . 266 

A. In adults 266 

B. In children — Cerebral palsy of children — Hemiplegia infantilis 

spastica — Polio-encephalitis 268 

Brain tumors 289 

Appendix — Parasites of the Brain 305 

Congenital diseases — Hydrocephalus — Meningocele — Porencephaly — 

Absence of certain parts of the brain 308 

Disease^ of the Spinal Cord 314 

PART I. 

Diseases of the Spinal Meninges 315 

Chap. I. — Inflammations of the dura mater — Pachymeningitis spinalis . .316 
II. — Inflammations of the soft spinal meninges — Leptomeningitis spinalis . 322 
III. — Haemorrhage into the spinal membranes — Meningeal apoplexy — Pachy- 
meningitis interna haemorrhagica ....... 326 

PART II. 

Diseases of the Spinal Nerves 330 

A. Diseases of the motor and sensory nerves 332 

I. Diseases of the cervical nerves 332 

Chap. I. — Lesions of the cervical plexus 336 

II. — Lesions of the brachial plexus ...... 340 

II. Diseases of the dorsal nerves 363 

III. Diseases of the lumbar nerves .' . . 366 

IV. Diseases of the sacral and coccygeal nerves . . . . . . 370 

V. Neuritis involving several spinal nerves at the same time — Multiple 

neuritis 387 

B. Diseases of the trophic and vaso-motor nerves ...... 397 

Appendix — Diseases of the muscles — Primary myopathies . . . 405 

PART III. 

Diseases of the Substance of the Spinal Cord 418 

I. Consideration of spinal diseases with reference to their seat — Topical diag- 
nosis 418 

I. Lesions of the gray matter — Poliomyelitis ...... 425 

Chap. I. — Poliomyelitis anterior acuta — infantile spinal paralysis . 426 
II. — Atrophia muscularis progressiva spinalis — Progressive 

muscular atrophy . 434 



CONTENTS. 



XI 



II. Lesions of the white matter of the spinal cord — Leucomyelitis . 

A. Primary lesions of the white columns 

B. Secondary lesions of the white columns 

in. Lesions of the gray and white matter of the spinal cord 

II. Spinal lesions regarded from their pathological aspect — Pathological diag- 
nosis 

I. Affections of the spinal cord due to diseases of the blood-vessels 

A. Diseases of the arteries of the spinal cord and their consequences 
i. Spinal haemorrhage — Hsemorrhagia (or apoplexia) medulla; 

spinalis — Hsematomyelia 

Embolism and thrombosis of the spinal arteries and myelo- 
malacia ...... 

Endarteritis (syphilitica) 
Dilatation of the spinal arteries . 
Neuroses of the spinal arteries 
Inflammatory processes in the substance of the spinal 
i. Purulent myelitis — Abscess of the spinal cord 
2. The non-purulent myelitis .... 

A. The acute form ..... 

B. The chronic form .... 
Spinal tumors . 

Appendix — Parasites of the spinal cord . 
Congenital diseases — Hydrorrhachis — Spina bifida 



cord 



PAGE 

439 
440 

445 
446 

458 
458 
458 

458 

460 

461 
462 
462 

405 
465 

4&5 
465 
467 
467 
470 
471 



Diseases of the General Nervous System 



476 



PART I. 

Diseases of the General Nervous System without any Recognizable 

Anatomical Basis — "Functional Neuroses" .... 479 
First Group. — Neuroses which are wont to run their course without any essen- 
tial implication of the general organism 481 

A. Affections in which the motor nerves are chiefly implicated . .481 
Chap. I. — Chorea — Chorea Sancti Viti — St. Vitus' dance — Ballismus — Mel- 
ancholia saltans — Sydenham's disease 481 

II. — Tetany — Tetanilla — Tetanus intermittens 493 

Thomsen's disease 496 

III. — Paralysis agitans — Shaking palsy — Parkinson's disease — Chorea 

procursiva 500 

B. Affections in which the sensory nerves are chiefly implicated . . 507 

Migraine — Hemicrania 507 

C. Affections in which the trophic nerves are chiefly implicated . .512 

1. Acromegaly 512 

2. Osteoarthropathy 516 

Appendix. 

1. Graves' disease — Basedow's disease — Exophthalmic goitre . . 518 

II. Myxoedema 5 2 5 

Second Group. — Neuroses in which the entire organism is more or less severely 

implicated . 529 

Chap. I. — Neurasthenia — Nervous prostration 529 

II. — Hysteria ' 539 



x ii CONTENTS. 



PAGE 



III. — Epilepsy — Falling sickness — Morbus sacer — Morbus comitialis . 571 
IV. — Hystero-epilepsy — Major hysteria — Hypnotism — Treatment by 

suggestion ........... 600 

PART II. 

Diseases of the General Nervous System with Known Anatomical 

Basis 616 

Chap. I. — Multiple sclerosis — Disseminated sclerosis — Insular sclerosis — Sclerose 

en plaques — Sclerosis cerebro-spinalis disseminata sive multiplex . 616 
II. — Tabes dorsalis — Locomotor ataxia — Posterior spinal sclerosis — Leuco- 

myelitis posterior chronica. ........ 629 

III. — Dementia paralytica progressiva — General paralysis of the insane — 

General paresis — Softening of the brain 688 

IV. — Syphilis of the general nervous system 700 



LIST OF ILLUSTRATIONS. 



FIG. 
I. 

2. 

3- 

4- 
5- 

6. 

7- 



9- 

10. 
11. 
12. 

13- 
14. 

15. 
16. 

17- 

18. 
19. 
20. 

21. 

22. 

23- 
24. 

25. 
26. 
27. 
28. 
29. 
30. 

3i- 

32. 
33- 
34- 



Gow 



albe) 
, and 



Cross-section through the cerebral cortex and its membranes 

Diagram showing the course of the optic fibres in the chiasm 

Diagram showing the origin of the optic nerve (after Wernicke) . 

Field of vision of the left and right eye (after Forster) . 

Field of vision of the left and right eye in left-sided hemianopia (after 
ers) 

Cross-section through the region of the ant. corpora quadrigemina 

Diagrammatic longitudinal section through the pons with the nuclei of the 
ocular nerves (after Gowers) . 

Cross-section through the region of the tegmentum (after Schwalbe) 

Cross-section through the pons (after Schwalbe) .... 

Nuclei of the trigeminal nerve (after Schwalbe) .... 

Cross-section through the medulla oblongata (after Schwalbej 

Distribution of the sensory cutaneous nerves on the head 

Diagram showing the course of the facial fibres in the pons (after Schw 

Diagram showing the decussation of the fibres going to the extremities 
those going to the face, in the pons and medulla oblongata 

Erb's diagram for facial paralysis ..... 

Some of the so-called " motor points " on the face and neck 

Diagrammatic section through the medulla oblongata in the region of the 
(lower) olive ........ 

Cross-section through the medulla oblongata (after Schwalbe 

Bilateral paralysis of the recurrent laryngeal . 

Recurrent laryngeal paralysis ...... 

Paralysis of the recurrent laryngeal on the left side 

Paralysis of both posterior crico-arytenoids . 

Paralysis of the right post, crico-arytenoid 

Paralysis of both internal thyro-arytenoids 

Paralysis of both internal thyro-arytenoids 

Cross-section through the cervical cord 

Superficial origin of the cranial nerves .... 

Cortical centres of the left hemisphere (after Gowers) . 

Hemiatrophia linguae 

Hemiatrophia linguae 

Pharyngeal and laryngeal electrode with arrangement for making and break- 
ing the current (after Erb) 

Facial expression in progressive bulbar paralysis (Leyden, Eichhorst) 

Cross-section through the upper portion of the medulla oblongata . 

The posterior (dorsal) aspect of the medulla oblongata . 

xiii 



4 

29 

3i 

37 

37 
42 

43 

44 
45 
57 
58 
74 
78 

84 
87 
93 

96 
in 
117 
117 
117 
117 
117 
117 
117 
136 
141 
142 

143 
144 

149 

154 
156 

157 



XIV 



LIST OF ILLUSTRATIONS. 



47- 

48. 
49. 



35. Right hemisphere (after Exner) 166 

36. Left hemisphere (after Exner) . 166 

37. Convolutions and fissures of the lateral aspect of the brain (after Ecker) . 167 

38. Convolutions and fissures at the base of the brain (diagrammatically, after 

Ecker) 168 

39. Diagram illustrating method of determining the location of the fissure of Ro- 

lando ............. 169 

40. Convolutions and fissures of the median aspect of the brain .... 170 

41. Convolutions of the island of Reil (J. R.) made visible by removing the oper- 

culum ... 170 

42. Topographical relations between the exterior of the skull and the surface of 

the brain (after Ecker) 171 

43. Wernicke's schema for the cortical mechanism of speech .... 175 

44. 45. Lichtheim's schema illustrating the seven different forms of aphasia . 179 
46. Diagram showing the direct system of fibres (Flechsig, Mendel) . . . 184 

Course of the fibres from the internal capsule to the crus cerebri (diagram- 
matic, after Wernicke and Edinger) 189 

View of the ventricles on horizontal section (after Edinger) .... 190 
Horizontal section through the brain, about a finger's breadth below that 

represented in Fig. 48 (Edinger) 191 

50-53. So-called " frontal sections " through the brain (after Edinger) . 192, 193 

54. Points at which the Pitres-Nothnagel sections are made .... 194 

55-60. Pitres-Nothnagel sections 195-197 

61. Diagrammatic cross-section through the anterior corpora quadrigemina (after 

Edinger) 200 

Longitudinal section through the region of the corpora quadrigemina of a 

human foetus twenty -eight weeks old (after Edinger) .... 201 
Diagrammatic horizontal section through the decussation of the superior pe- 
duncles of the cereDellum (after Edinger) 201 

Sagittal section through pons and medulla oblongata (after Mendel) . . 202 

Cross-section through the region of the ant. corpora quadrigemina . . 203 

Diagram showing the decussation of the fibres going to the extremities, and 

of those going to the face, in the pons and medulla oblongata . . 204 

The connections of the cerebellum 207 

Diagram showing the circle of Willis 210 

69. The cortical distribution of the middle cerebral artery (after Charcot) . . 211 

70. Frontal section through the cerebral hemispheres, one centimetre behind the 

chiasm 212 

Cerebral artery from an apoplectic focus (after Cornil and Ranvier) . . 213 

Miliary aneurism of a small artery of the lenticular nucleus (after Marchand) 214 

The large head electrode (covered with sponge) of Erb ..... 241 

Porencephaly 267 

Hemiatrophy of the left side of the body, front 274 

Hemiatrophy of the left side of the body, back 275 

Hemiatrophy of the left side of the body from traumatism . ... . . 276 

78. Hemiatrophy of the left side of the body from traumatism .... 277 

79. Atrophy of the left upper and lower extremity 278 

80. The family form of spastic paraplegia (after Newmark) 279 

81. Atrophy of paralyzed side 280 

82. Atrophy of paralyzed side ; contracture of wrist 281 

83. Atrophy of paralyzed side ; contracture of ankle ...... 282 

84. Atrophy of paralyzed side ; contracture of ankle ...... 283 



62. 

63. 

64. 
65. 
66. 

67. 

68. 



71. 

72. 

73- 
74- 

75- 
76. 

77- 



LIST OF ILLUSTRATIONS. XV 

FIG. PAGE 

85. Convulsive movements of the extremities 285 

86. Glioma telangiectaticum (after Ziegler) 289 

87. Papillary carcinoma in the third ventricle (after Ziegler) .... 291 
88.-Cysticercus racemosus (after Marchand) 305 

89. Hydrocephalus 309 

90. Cross-section through the vertebral column and the spinal cord (diagram- 

matical) (after Eichhorst) 316 

91. Cross-section through the middle of the cervical enlargement in pachymen- 

ingitis cervicalis hypertrophica (after Charcot) 317 

92. Position of the hand in pachymeningitis cervicalis hypertrophica (Charcot) . 319 

93. Diagrammatic outline of the cervical and brachial plexuses (after Schwalbe) 333 

94. Case of right-sided serratus paralysis in a man thirty- five years of age (after 

Eichhorst) 341 

95. The same case with the arms raised 342 

96. Position of the head in spasm of the splenius capitis on the right side . . 343 

97. Musculo-spiral paralysis 344 

98. Motor points of the musculo-spiral nerve and the muscles supplied by it . 347 

99. 100. The distribution of the cutaneous nerves of the arm and hand (after 

Eichhorst) 348 

101. Distribution of the sensory nerves on the back of the fingers (Krause) . . 349 

102. Motor points of the median nerve and the muscles supplied by it . .350 

103. Motor points of the ulnar nerve and the muscles supplied by it . . . 350 

104. Motor points of the ulnar nerve 351 

105. Claw-hand (after Duchenne) 352 

106. Motor points of the musculo-cutaneous nerve and the muscles supplied by it 352 

107. Motor points of the brachial plexus ; Erb's supraclavicular point . . . 355 
108-111. The manner in which a child whose erectores spinas are paralyzed gets 

up from the ground (after Gowers) ....... 366 

112. Diagrammatic outline of the lumbar and sacral plexuses .... 367 

113, 114. Areas of distribution of the cutaneous nerves of the lower extremity 

(after Henle) 368 

115. Motor points for the nerves and muscles of the anterior surface of the leg . 382 

116. Motor points for the sciatic nerve and the muscles supplied by it . . 383 

117. Case of peripheral neuritis of the sciatic nerve, with shortening and atrophy 

of the affected extremity 384 

118. Case of peripheral neuritis of the sciatic nerve, with shortening and atrophy 

of the affected extremity ......... 385 

119. 120. Contracture in the quadratus lumborum 386 

121. Atrophy of the muscles of the right upper arm in consequence of a fracture 

of the humerus seven years previously . 389 

122, 123. Panarthritis with secondary multiple neuritis .... 390, 391 

124. Hemiatrophia facialis ........... 404 

125. So-called juvenile muscular atrophy (Erb) 407 

126. Juvenile muscular atrophy (Erb) . ........ 408 

127. Juvenile muscular atrophy (Erb) 4°9 

128. Juvenile muscular atrophy (Erb) 4 10 

129. Progressive atrophic myopathy (after Marie et Guinon) . . " .411 

130. Pseudo-hypertrophy of the muscles of the legs, with atrophy of the muscles 

of the back (after Duchenne) 4 I 3 

131. Absence of the forearms 4*4 

132. The relations of the origin of the nerves to the bodies of the vertebrae and 

the spinous processes (after Gowers) 5*9 



xvi LIST OF ILLUSTRATIONS. 

FIG. PAGE 

133. Scheme of the conducting paths in the spinal cord at the level of fifth dorsal 

nerve (after Flechsig) . . 420 

134. Cross-section through the spinal cord at different levels (after Quain) . . 420 

135. Reflex arc 421 

136. Transverse section from the cervical portion of the spinal cord (after 

Charcot) 426 

137. Spinal infantile paralysis 427 

138. 139. Progressive muscular atrophy (after Eichhorst) 435 

140, 141. Progressive muscular atrophy 436, 437 

142. Friedreich's disease (after Chauffard) . . 443 

143. Ascending and descending degeneration in the spinal cord (after Gowers) . 446 

144. Secondary ascending and descending degeneration in a transverse affection 

of the upper dorsal cord (after Strumpell) 446 

145. 146. Complete interruption of conduction of the spinal cord during life (after 

Eichhorst) 454 

147. Schema of the course of the nerve fibres in the spinal cord (after Brown- 

Sequard) 457 

148, 149. Thomsen's disease (after Mills) 497, 498 

150, 151. Specimens of handwriting of patient with paralysis agitans . . 501, 502 

152. Position of hands and fingers in paralysis agitans (after Eichhorst) , . 503 

153. Position of the body in paralysis agitans . 504 

154. Enlargement of jaw in acromegaly (after Marie) 512 

155. Case of acromegaly (after Marie) 513 

156. Case of acromegaly (after Buchwald) 514 

157. Osteoarthropathy (after Rauzier) 516 

158. Osteoarthropathy (after Spillmann and Haushalter) 517 

159. Graves' disease 519 

160. Myxcedema (after Charcot) 526 

161. " Idiotie myxoedemateuse " .......... 527 

162. 163. Hysterical muscular atrophy ........ 546, 547 

164. Specimen of handwriting in a case of multiple sclerosis .... 617 

165. Specimen of handwriting illustrating alcoholic tremor ..... 622 

166. Specimen of handwriting illustrating tremor senilis 623 

167. Specimen of handwriting of a patient with mercurial tremor . . . 624 

168. Specimen of handwriting illustrating the tremor produced by the combined 

action of alcohol and mercury 625 

169. Cross-section through the cervical enlargement of the spinal cord in a case 

of multiple sclerosis (after Bramwell) 626 

170. Hemiatrophy of the tongue in an otherwise perfectly healthy child . . 637 

171. Specimen of handwriting in a case of tremor 111 tabes ..... 643 

172. Two cases of tabes (after Westphal) 649 

173. A case of Charcot's joint in a tabetic 654 

174. Erosion of the head of he humerus in tabes dorsalis (after Charcot) . . 656 

175. Normal humerus (after Charcot) 656 

176. Skeleton of a tabetic foot (after Charcot) 657 

177. Plantar flexion of the toes in the course of tabes 661 

178. Section through the cervical cord in a case of commencing tabes (after 

Strumpell) 673 

179. Section through the lumbar cord in tabes (after Strumpell) .... 973 

180. Section through the cervical cord in a case of advanced tabes (after Strum- 

pell) 673 

181. Suspension apparatus used in the treatment of tabes 685 



DISEASES OF THE BRAIN AND ITS MENINGES, 
INCLUDING THE CRANIAL NERVES. 



The study of brain diseases, we must confess, has not made 
the strides that might have been expected after the numerous 
and varied researches that the last decades have seen. For 
this our present very imperfect knowledge of the anatomy, and 
still more our doubts as to the physiological functions of the 
different parts of the brain must be held largely responsible. 
The structure as well as the physiological functions of the 
human brain are, up to the present time, so little understood 
that we are far from having any sure basis upon which to lay 
the foundations of a cerebral pathology. No small progress 
has been made from an anatomical standpoint through Sel- 
ling's method of serial sections, a method which Meynert, 
Henle, Wernicke, and others have not been slow to use, in 
their admirable researches, to which important additions have 
been made by the embryological studies of Flechsig, and by 
the method of " arrested development " used by Gudden and 
his pupils (atrophy method; Degenerationsmethode, Schwalbe); 
but with all this we have only here and there single stones 
which we have not as yet been able to combine for the con- 
struction of a harmonious whole. Brilliant from a physiologi- 
cal standpoint as was the discovery of Fritsch and Hitzig (1870) 
of the electrical irritability of the cortex, and of the existence 
of motor regions therein, unexpected as were the results which 
the experimental method of Munk brought to light, extraordi- 
nary and interesting as are the conclusions based upon the clini- 
cal and post-mortem observations of Charcot and his school — 
all these, wide-reaching and admirable as they were, are far 



2 DISEASES OF THE BRAIN. 

from having given us a full understanding of the functions of 
the different parts of the brain, and an explanation of the dis- 
turbances to which they are subject. Constant and untiring 
work is still needed, and the best results are promised from the 
intelligent combination of clinical observation with pathologi- 
cal research. The pathology of the brain can not be better 
advanced than by the patient clinical observation of cases dur- 
ing life and a careful autopsy after death. In institutions 
where not only the fullest opportunities are afforded for clin- 
ical observation and for the systematic conduct of post-mortem 
examinations of the brain, but where also the best men are 
found to supervise the work, in these will cerebral pathology 
make the greatest strides. 

We shall divide our description of cerebral diseases into 
three parts. In the first we shall take up the diseases of the me- 
ninges, in the second those of the cranial nerves, while the third 
will embrace the diseases of the brain in the stricter sense, i. e.,. 
those of the white and gray matter of the hemispheres and of 
the central ganglia. 



PART I. 

DISEASES OF THE MENINGES OF THE BRAIN. 

The meninges are relatively more frequently affected by 
disease than the brain substance itself, and quite a consider- 
able number of the cases which we commonly call diseases of 
the brain are really to be classed as affections of the meninges. 
Since these diseases can develop under the most varied con- 
ditions, and can be primary as well as secondary, they are of 
great practical importance, and we must try to distinguish 
most carefully between the different forms which they assume. 

A clear understanding of the pathological processes in these 
diseases will be facilitated by some remarks upon the anatomy 
of the meninges. 

The outermost, tough, fibro-tendinous membrane, called the dura 
mater, forms at the same time the inner periosteum of the cranial 
bones. It has an outer, rough, and an inner, smooth surface. For 
the nerves as they emerge from the skull this membrane supplies 
sheath-like coverings, among which that of the optic nerve (vagina 
optici) is the most conspicuous. The blood-supply of the dura is de- 
rived from branches of the meningeal arteries. That it possesses its 
own nerves is doubted by some (among them Luschka), affirmed by 
others (Ruedinger, Alexander). It is most probable, however, that 
it is the trigeminus which chiefly provides for the innervation of the 
dura. 

The second membrane, the arachnoid, is delicate and contains no 
vessels. Its outer surface is smooth and looks toward the subdural 
space, while the inner is rough and turned toward the pia mater. 
The so-called subarachnoid space (Fig. i), which is situated between 
the arachnoid and pia, contains between the meshes of the subarach- 
noid tissue the serous cerebro-spinal fluid. 

The third membrane, the innermost, the one which lies directly 
on the surface of the brain, is called the pia mater. It dips down 
into the depths of the sulci, forming a continuous lining of those 
parts of the brain-stem which are covered by the cerebrum and 

3 



DISEASES OF THE MENINGES OF' THE BRAIN. 



cerebellum, and seems to penetrate through the so-called fissures 
into the interior of the brain. These processes, which are called 
telae choroidese, present peculiar villous formations, very rich 

in capillary vessels, 
sa and therefore of 

a deep - red color 
(plexus choroidei). 
The covering or 
ependyma of the 
ventricles is not a 
part of the pia ma- 
ter, but is simply a 
layer of epithelial 
cells. The nerves 
of the pia mater 
belong to the sym- 
pathetic. 

The diseases 
of the meninges 
of the brain con- 
sist mainly of in- 
flammatory processes affecting either the pia or the dura mater. 
We shall study the diseases of the two membranes separately. 




Fig. i. — Cross Section through the Cerebral Cortex 
and its Membranes. 

co, Cortex ; p, pia mater ; s. a, subarachnoid space ; s. d, sub- 
dural space ; d, dura mater ; v. v, blood vessels. 



CHAPTER I. 



INFLAMMATION OF THE INNER SURFACE OF THE DURA MATER, 
PACHYMENINGITIS INTERNA HEMORRHAGICA, HEMATOMA DURE 
MATRIS. 

The origin of the extravasations of blood which at the 
autopsy are often found on the inner surface of the dura, and 
which can be easily scraped off w T ith the knife, is not alto- 
gether understood. Some (Virchow, 1856) hold that the pri- 
mary affection is an inflammation, and the haemorrhage takes 
place secondarily into the newly formed, highly vascular con- 
nective tissue, while others look upon the haemorrhage as pri- 
mary ; and, indeed, recent observations (Sperling) seem to be 
very much in favor of this latter view. If extensive haemor- 
rhages occur, after spreading over more or less of the inner 
surface of the dura they become encapsulated, and are then 



PACHYMENINGITIS INTERNA HEMORRHAGICA. r 

called hcematomata durce matris. Such a haematoma may con- 
tain from three hundred to four hundred grammes of extrava- 
sated blood, may attain the size of a man's fist, and so exert 
a deleterious pressure upon the brain. The walls are some- 
times smooth, sometimes rough ; the contents are not always 
sanguineous, but may be serous or purulent. They are most 
commonly situated at the vertex near the falx cerebri, some- 
times also in the frontal region, very rarely at the base. The 
arrangement of the hasmatoma in layers, which is seen on sec- 
tion, proves that the whole process consists of extravasations 
which have occurred at different times. In the least-marked 
cases only a delicate reddish membrane is found, presenting 
reddish or brownish specks, and is easily stripped off from the 
dura. Only gradually the different layers are developed, the 
one nearest to the brain, of course, being always the most 
recent, the one lying on the dura the oldest. Between the 
layers are the haemorrhages. If it happens that the most re- 
cent layer is perforated by the haemorrhage there occurs free 
extravasation of blood between the dura and the arachnoid — 
that is, an intermeningeal haemorrhage. 

iEtiology. — In the aetiology, diseases of the heart and kid- 
neys, but especially chronic diseases of the brain, play by far 
the most important part. The lesion is seen in almost all affec- 
tions which lead to an atrophy of the brain ; further, it may be 
met with in infectious diseases — for instance, in typhoid fever, 
scarlet fever, acute rheumatism ; also in conditions of what we 
may call blood-dissolution, as in the general haemorrhagic di- 
athesis. Frank C. Hoyt, of New York, has called attention in 
this connection to a lowering or complete paralysis of the vaso- 
motor tone, which according to him is associated with struc- 
tural changes in the blood-vessels (Medical Record, 1892, 41). 
Among the exciting causes are traumatism of the cranial bones 
and inflammation in the neighboring parts — for instance, in the 
petrous portion of the temporal bone. Of predominating im- 
portance, as an aetiological factor, is the abuse of alcohol. Al- 
most in all autopsies on old drunkards we find a more or less 
well developed pachymeningitis interna, which has recently 
also been experimentally produced in dogs by continued doses 
of alcohol (Leyden). The fact that statistics have established 
that men, and more especially old men, are by preference 
affected by this disease also seems to point to alcohol as the 
principal cause. 



6 DISEASES OF THE MENINGES OF THE BRAIN. 

Symptoms may be entirely absent. This is the case when 
the haemorrhage, or the newly formed membranes are not of 
sufficient extent ; but if symptoms are present, then among the 
most important we find headache, which may persist for years, 
but which of course in itself, even if we have a history point- 
ing to this disease, as, for instance, the abuse of alcohol, is never 
sufficient to justify the diagnosis. With a sudden rise of intra- 
cranial pressure we always have apoplectiform attacks, in 
which consciousness is lost for a variable time, and in which 
the patient may die without regaining consciousness. Vomit- 
ing, slow pulse, and a very conspicuous narrowing of the pupil 
are not wont to be absent. Repeatedly peculiar dreamy con- 
ditions have been observed after such a coma, during which 
the patients seem completely dazed and the urine and faeces 
are passed involuntarily. If the haematoma lies over the mo- 
tor area, epileptiform convulsions and hemiplegia may result, 
serious motor disturbances, limited to one side, which may en- 
tirely disappear in a short while, or may last for months. Uni- 
lateral nystagmus and choked disk have been reported by some 
(Fuerstner). The further course depends upon the absorption 
of the clot or the occurrence of a further haemorrhage, as the 
case may be. The repeated development of severe cerebral 
symptoms, after striking and rapid improvement, speaks under 
certain circumstances for the existence of haematoma of the 
dura, because it is just this frequent change in the condition of 
the patient which is characteristic of the course of the disease. 
Months and even years may thus pass without a fatal result, 
and much more rarely than one would be led to expect is it 
possible to make a positive diagnosis during life, because all 
the symptoms which we have mentioned can be found just 
as well in other cerebral affections, in haemorrhage, embolism, 
new growths, etc., and the only thing we have to fall back upon 
is the history, if this be one of alcoholic excesses. The parox- 
ysmal appearance of new symptoms is not to be overlooked, 
inasmuch as it confirms to some extent the diagnosis of pachy- 
meningitis. However, under all circumstances the task is a 
difficult one. The cases described by French writers (e. g., 
Puech, Progres medical, 1889, 17) under the name apoplexie pro- 
gressive are instances of this affection. 

Prognosis. — The prognosis for recovery is of course abso- 
lutely bad if thickening has reached any degree worth men- 
tioning ; and when we have to deal with a large haematoma 



PACHYMENINGITIS INTERNA HEMORRHAGICA. j 

which encroaches considerably upon the intracranial space the 
prospect for life is, to say the least, not hopeful. On various 
anatomical grounds death can occur suddenly and unexpect- 
edly.. 

Treatment can only be of any value in the earlier stages, 
but unfortunately the disease is usually not recognized then. 
Interdiction, or at least restriction, of the use of alcohol, if this 
plays a part, energetic antiphlogistic treatment in the form of 
local bloodletting, the ice-cap to the head, counter-irritation by 
inunctions of mercurial ointment, and active purgation (calo- 
mel) would surely give us good results ; but, as we have said, 
these means are, as a rule, used too late, and as a matter of 
fact the progress of the disease is usually not altered by any 
therapeutic measures. 

The most common new growths of the dura mater are 
sarcomata (endothelioma, fungus duras matris) or osteomata. 
Fibromata and lipomata are but rarely met with. They are 
only of pathological and not of clinical interest, since they do 
not give rise to typical symptoms. 

LITERATURE. 

Eulenburg, A. Lehrbuch der Nervenkrankheiten. 2. Aufl. Berlin, 1878. 

Ziegler. Lehrbuch der allgemeinen und speciellen patholog. Anatomic Jena, 
1882. 

Wernicke. Lehrbuch der Gehirnkrankheiten. Bd. iii, pp. 483 et seq. Berlin, 
1883. 

Gowers. Lectures on the Diagnosis of Diseases of the Brain, delivered at Uni- 
versity College Hospital, 1885. Churchhill ; also Blakiston, Philadelphia. 

Liebermeister. Vorlesungen iiber die Krankheiten des Nervensystems. P. 306 
et seq. Leipzig: Vogel, 1886. 

Eichhorst. Handbuch der speciellen Pathologie und Therapie. Bd. iii, pp. 439 
et seq. 3. Aufl. Wien und Leipzig, 1887. 

Seeligmiiller. Lehrbuch der Krankheiten des Riickenmarks und Gehirns. Abth. 
II, pp. 401 et seq. Braunschweig, 1887. 

Hoffmann. Zur Pathologie und Therapie der Pachym. ext. purul. nach Ent- 
ziindungen des Mittelohres. Deutsche Zeitschrift fur Chirurgie, May 4, 
1888, vol. xviii. 

Thiroloix et du Pasquier. Ossification de la dure mere ; mort par hemorrhagic 
cerebrale. Bull, de la Soc. anat., Jan. 2, 1892, 5 sen, vii. 

Willett. Fibroma of the Dura Mater without Symptoms. Path. Soc. Transac- 
tions, 1892, xliii, p. 6. 



CHAPTER II. 

INFLAMMATIONS OF THE SOFT MEMBRANES OF THE BRAIN ; LEPTO- 
MENINGITIS ; PURULENT MENINGITIS. 

A. Pathological Anatomy. ^Etiology. 

Inflammations of the soft cerebral meninges occur either 
at the base or at the convexity of the brain, according as they 
are primary or secondary — i. e. y associated with other diseases- 
— and one can, indeed, with a few exceptions and bearing in 
mind the transition forms, put it down as a rule that secondary, 
metastatic meningitis affects the convexity, while a primary 
meningitis is usually found at the base. 

In contradistinction to what takes place in the dura, where 
the only purulent inflammations that we find are such as have 
extended by contiguity from neighboring parts, here we have 
to deal with purulent inflammations alone. This purulent in- 
flammation of the soft membranes of the brain, the leptomenin- 
gitis cerebralis, is an infectious disease, and occurs in epidemics 
as epidemic cerebro-spinal meningitis, or more rarely sporadi- 
cally, the two forms, however, being aetiologically identical. 
Besides these, we find developing in the course of tuberculosis, 
sometimes very early, sometimes late, a specific form of menin- 
gitis, the tubercular meningitis. 

Pathological Anatomy. — The pathological processes can be 
traced in the pia as well as in the substance of the brain. In 
the meshes of the former we find a purulent exudate, which is 
in rare cases limited to one hemisphere ; if it is copious, the pia 
can easily be stripped from the brain ; if it is scanty, this can 
not be done without loss of substance. The brain substance is 
©edematous and fills up the skull more than normally, so that 
the convolutions appear flattened. The ventricles are filled 
with an unusual amount of fluid (hydrocephalus internus). The 
haemorrhages which are recognizable in the brain substance do 
not exceed in size that of a pin's head, and are either isolated- 



LEP TOMENINGI TIS. g 

or are seen especially near the ventricular walls in greater 
numbers, the so-called capillary apoplexies. Besides these 
there are other small punctiform haemorrhages, or rather spots 
of red softening, and minute haemorrhages closely grouped to- 
gether. All these focal changes are to be looked upon as due 
to the influence of the specific virus. If the process has be- 
come a chronic one, then the characteristic features are oedema 
of the pia, wasting of the brain substance, hydrocephalus inter- 
nus, and thickening of the ventricular ependyma, which gives 
to the surface a velvety appearance and changes the shape of 
the ventricles in a characteristic manner, the normally sharp 
edges becoming rounded off (chronic meningitis). 

In tubercular meningitis we find not only signs of an in- 
flammatory process, but also the formation of tubercles ; both, 
however, do not progress pari passu. There may be a very 
extensive eruption of tubercles and a relatively slight inflamma- 
tion, or vice versa, but always, especially in children, the greater 
part of the jelly-like exudate is situated at the base (basilar 
meningitis), between the pons and the anterior perforated 
space, and imbedded in it are the grayish- white tubercles which 
are seen as nodules, sometimes as large as millet-seeds, and are 
found in the greatest numbers among the larger vessels of the 
fissure of Sylvius, on the chiasma, pons, etc. The vessels are 
fuller than usual, and small haemorrhages can occasionally be 
seen in the pia. The substance of the brain is affected in the 
manner above mentioned — hydrocephalic effusions into the ven- 
tricles are rarely absent, and there is a decided fullness of the 
choroid plexuses. Foci of softening are noted chiefly about the 
basal ganglia ; they are produced sometimes by the occlusion of 
an artery, sometimes by the pressure which the exudate exerts 
on the vessel, or, again, by an arteritis obliterans. Regenera- 
tion has been known to occur even in tuberculous meningitis. 
Dilatation of the ventricles and other signs of an increased 
intracranial pressure may continue, and collections of fluid in 
the pia and in the ventricles may still be present, but the fluid 
may again become clear, the pia moist and non-adherent to the 
cortex, and the tubercles present no inflammation around them 
(Wernicke). 

A chronic form of basal meningitis, in which the pia is in 
places either thickened and indurated, or where we have a 
formation of brittle crusts, may be of a gummatous nature 
(Wernicke). When a purulent process in the dura extends 



IO DISEASES OF THE' MENINGES OF THE BRAIN. 

into the sinuses we get what is called a thrombo-phlebitis or a 
(marantic) sinus thrombosis (see Diseases of the Cerebral Veins). 

-^Etiology. — As has been stated, cerebro-spinal meningitis 
has to be looked upon as an infectious, sometimes epidemic, 
disease, the parasitic nature of which was demonstrated by 
Leyden in 1883. He found in the tissues of the pia and in 
the turbid cerebro-spinal fluid diplococci, which A. Fraenkel 
(Deutsche medicinische Wochenschrift, 1886, 13) and G. Hauser 
(Miinchener med. Wochenschr., 1888, 36) recognized as identical 
with the pneumococcus. Whether or not these cocci gain 
access to the meninges through the nasal cavities and the fo- 
ramina of the ethmoidal plate, we are unable to say. Children 
and young people are more easily affected by the disease than 
adults, and the infection can be carried by them from place to 
place. In inclosed and crowded localities, e. g., in prisons and 
barracks, the disease may become endemic. 

But even when there is no epidemic, the disease may ap- 
pear sporadically anywhere, and then also must be regarded as 
being just as much of a parasitic nature. Whether the direct 
influence of the sun's rays is capable of producing meningitis, 
or at least of favoring its development, has not thus far been 
sufficiently studied. 

A tangible cause for meningitis we find in traumatism of 
the cranial bones, causing injury to the soft parts, so that the 
pathogenic organisms can penetrate through the open wounds. 
The (septic?) Streptococcus pyogenes (Eberth), which is less deli- 
cate and more resistant than the above-mentioned coccus, has 
been demonstrated in such cases. If, however, in traumatism, 
the air remains excluded, as happens in fractures at the base, 
then the presence of a purulent meningitis is difficult to explain. 

The diseases of the bones of the skull, more especially those 
of the petrous portion of the temporal bone and of the auditory 
apparatus, play an important part in the astiology of meningitis. 
From an otitis media may be developed a caries of the petrous 
portion of the temporal bone which may perforate the thin 
roof of the tympanic cavity. The infection extends in such 
cases along the auditory nerve (Kirchner, Berliner klin. Wo- 
chenschr., 1893, 23). Another extension of the inflammation 
may come from the mastoid cells if an embolus passing from 
the veins of the bone lodges in one of the venous sinuses, 
which then becomes the seat of a purulent thrombo-phlebitis. 

That the tuberculous meningitis has its origin in tubercu- 



LEP TOMENINGI TIS. 1 1 

lous processes in other organs is clear, and the aetiology is 
therefore identical with that of tuberculosis in general — i. e., 
there is invariably an invasion by the tubercle bacillus. It is 
an interesting fact, however, that though the primary disease 
in other organs need not necessarily have produced any or at 
least no marked disturbances, we can still have secondary 
disease of the pia with the symptoms peculiar to it, which we 
shall describe. Children especially are not rarely attacked by 
meningitis, the tuberculous nature of which is only recognized 
at the autopsy, and we may not have the faintest suspicion of 
the existence of a previous tuberculous infection. In other 
cases, however, the meningitis only appears after the pulmonary 
tuberculosis has made great progress. Caseous bronchial and 
mesenteric glands, as well as solitary tubercles in the brain, 
may be the starting point of the meningeal affection, while it less 
commonly follows tuberculosis of the joints or bones, or tuber- 
culous affections of the intestines and genito-urinary apparatus. 

The relation of meningitis to other diseases — i. e., its simul- 
taneous appearance with influenza, pneumonia, scarlet fever, 
and typhoid fever, ulcerative endocarditis, etc. — has been care- 
fully studied by Huguenin (Correspondenzblatt fur Schweizer 
Aerzte, 1890, 23, 24), but the question whether in those cases 
we have to deal with a double infection, or whether we have 
a single noxious agent which produces both the meningitis 
and the affection which accompanies it, deserves further study. 
F. Wolff has recently discussed the possible relation of the 
occurrence of cerebro-spinal meningitis to meteorological con- 
ditions — e. g., to the degree of humidity in the atmosphere. 
The fact that so many cases occur between February and June 
is perhaps to be regarded as a consequence of the greater 
humidity which commences in September and does not de- 
crease until April ; scarcely any cases occur in July and 
August, during the period of atmospheric dryness which com- 
mences in May (Deutsch. med. Wochenschr., 1888, 38). 

It is well established that children and young people are 
more frequently and more severely attacked by meningitis than 
older persons, and it seems as if the disease is never found in 
old age. Early childhood, the period between two and three 
years of age, furnishes relatively the greatest number of vic- 
tims and gives the most unfavorable outlook (cf. Kohts, Ueber 
Paralysen und Pseudoparalysen im Kindesalter nach Influenza, 
Therapeut. Monatshefte, 1890). 



12 DISEASES OF THE MENINGES OF THE BRAIN. 

B. Symptoms, Diagnosis, and Treatment. 

Symptoms. — The idiopathic, purulent meningitis of the 
adult usually begins after insignificant prodromal symptoms, 
such as digestive disturbances, hebetude, etc., with headache, 
which soon attracts by its severity and its duration the atten- 
tion of the physician. Exceptionally the patient has hours of 
comparative ease ; usually the headache is so intense that he 
becomes almost frantic. He tosses about in bed with sighs 
and groans, and, even when the mind has become dulled, in- 
voluntarily again and again puts his hand to his head. Some- 
times delirium develops early, to cease again and sooner or 
later give way to a dull and somnolent condition, which in its 
turn passes into a deep coma, the immediate forerunner of death. 

In some cases the diagnosis is facilitated by characteristic 
symptoms, such as rigidity of the neck and marked hyper- 
esthesia of the skin and muscles. The former is especially 
well recognizable when the patient is asked to sit up in bed, 
which he can not do without intense pain ; the latter is often 
detected in the examination of the patellar reflexes, which 
themselves present no particular abnormalities. If we then 
find besides these symptoms in the beginning of the disease 
occasional (cerebral) vomiting, a strikingly slow pulse, which is 
in remarkable contrast with the elevation of temperature (102 
and more), and if we carefully examine the pupils, we can not 
easily make a mistake in the diagnosis. The pupils are usually 
very much contracted, but may show alternate contraction and 
dilatation when illuminated for any length of time (Oestreicher, 
Paradoxe Pupillenreaction, Berl. klin. Wochenschr., 1890, 6). 
Only exceptionally, however, do we meet with a combination 
so favorable for the task of the diagnostician. More frequently, 
as we shall explain at length, he has to encounter considerable 
difficulties. There is no doubt but that the vomiting is of 
cerebral origin ; but where the center for this is to be sought, 
whether in the medulla oblongata or, as Hlasko claims (Dor- 
pat, Inaugural Dissertation, 1887), in the corpora quadrigemina, 
still remains undecided, as also does the question whether or 
not we are dealing with a functional stimulation of this center. 
Choked disk and transient paralysis of the ocular muscles are 
occasionally observed. The former is not easily recognized 
when the patient quickly passes into sopor ; the latter, how- 
ever, is recognized without difficulty by the strabismus which 



LEP TOMEXIXGITJS. 



13 



it causes and the nystagmus-like movements of the eyeballs. 
Symptoms of irritation, partly referable to the cortex, in the 
form of general or unilateral convulsions, muscular unrest, or 
carphology, partly to individual cranial nerves (grinding of 
the teeth, trismus, facial spasm), have been repeatedly noted. 
They seem, however, not always to occur, and for diagnosis 
must be considered as of minor importance. 

The course of purulent meningitis in the adult is different 
in different cases. As a general rule, however, certain symp- 
toms, especially headache and the rigidity of the neck, some- 
times hyperassthesia of large areas of the skin, persist from the 
onset and increase, while others, as, for instance, the vomiting 
and the cranial nerve symptoms, are only transient. 

The duration of the disease can be two, three, four, to eight, 
more rarely ten to fourteen days, and the younger the patient 
the more dangerous is usually the disease. The patients die, 
as a rule, without regaining consciousness, but the coma may 
last for days. 

The symptoms of the epidemic contagious (Kohlmann, 
Berliner klin. Wochenschr., 1883, 17) cerebro-spinal meningitis 
are on the whole quite similar to those of the idiopathic form. 
In both the headache is the predominating symptom, and the 
rigidity of the neck is rarely absent, but in the epidemic more 
frequently than in the idiopathic form the disease begins with 
a chill. The course of the fever presents nothing character- 
istic. It is sometimes of a remittent, sometimes of an inter- 
mittent type, the temperature sometimes reaching a height of 
104 to 107 F. More or less severe disturbances of con- 
sciousness may occur even without a marked elevation of tem- 
perature. Among the cranial nerve symptoms, the disturb- 
ance in hearing caused by the auditory nerve taking part in 
the inflammatory process has to be mentioned (Schwabach, 
Zeitschr. f. klin. Med., 1891, xviii, 3, 4). Visual disturbances 
are more uncommon, but optic neuritis has been repeatedly 
noted. If other cerebral symptoms — convulsions, hemiplegia, 
aphasia — occur, they have to be considered as complications 
due to an extension of the inflammation to certain parts of the 
brain substance. 

The spinal symptoms, which are superadded, may consist 
of a distinct tenderness along the whole vertebral column, of a 
hyperesthesia of the legs (which is of diagnostic importance), 
and of twitchings of the extremities. A peculiar, but, as it 



14 



DISEASES OF THE MENINGES OF THE BRAIN. 



appears, extremely uncommon symptom is the so-called flexor 
contracture of Kernig : the patient when in a sitting posture 
is unable to extend his knees, because a contracture in the 
flexors is developed, which disappears as soon as the thigh is 
no longer flexed at the hip-joint. Bull (cf. lit.) has made some 
communications on this point. The mechanism of micturition 
is only influenced when the patient becomes unconscious ; then 
the urine is passed involuntarily. Besides this there are no 
important bladder symptoms. The urine sometimes contains 
albumin or sugar, also some tube-casts. Sometimes the quan- 
tity voided is greatly increased, a polyuria, which we have to 
consider as a cerebral symptom. 

Other organs rarely take part in the disease. The circula- 
tory, respiratory, and digestive apparatus usually remain nor- 
mal, and serious stomach affections, endocarditis, and pneu- 
monia, of which we have already made mention above, are 
seen only rarely as complications. Moderate splenic enlarge- 
ment often occurs. Among the skin eruptions which some- 
times accompany cerebro-spinal meningitis, besides urticaria 
and (much more rarely) roseola, we have a herpes labialis, 
which, without being of any prognostic value, possesses a cer- 
tain diagnostic significance. 

The course of epidemic meningitis is still more uncertain 
and variable than that of the idiopathic form. It may be rapid, 
and end fatally within a day or a day and a half, in which case 
convulsions are followed by deep and persistent coma. It 
may, however, also be protracted, and with remissions, during 
which the patient is in fairly good condition, may last for 
weeks. In the beginning of the epidemic usually grave cases 
are more common, while the longer it lasts the milder they be- 
come. It seems as if an attenuation in the virulence of the mi- 
croorganism had taken place. There occur, besides, abortive 
cases, in which, while they undoubtedly must be classed with 
the epidemic disease, only a small, sometimes quite insignifi- 
cant, part of the symptoms are developed. The period of in- 
cubation is from three to five days. Frequently an attack of 
cerebro-spinal meningitis is followed by certain sequelae, among 
the most common of which are headache, pain in the neck, or 
neuralgias, which may persist for a long time after convales- 
cence. 

The symptoms of tuberculous meningitis differ somewhat 
in children and in adults. 



LEP TOMENINGITIS. 



15 



(a) In children the disease runs either a very acute or a more 
chronic course. In the first case only a few days may elapse 
between the onset and the fatal issue ; in the latter, weeks and 
months may pass before amelioration and recovery, or in these 
cases also death takes place. 

The acute form usually begins suddenly with epileptiform 
convulsions. Apparently healthy, robust children fall into con- 
vulsions and then complain of severe headache and nausea, 
which is often followed by vomiting ; the pulse becomes irreg- 
ular, and its variations in frequency are more striking than in 
any other disease. On examination, we find the temperature 
only moderately elevated, but the patient is very restless, 
throwing himself about in bed and complaining of pain in the 
abdomen, chest, etc. Strabismus, trismus, grinding of the 
teeth, are often noted, and on mechanical stimulation of the 
skin striking circumscribed red spots, Trousseau's taclics cere- 
brates, appear. The patients sigh deeply when examined, or 
give an unexpected loud, sharp cry, the cri hydrence'phalique, a 
very unfavorable symptom which is of far greater importance 
than the spots, from the appearance of which we are not justi- 
fied in drawing either favorable or unfavorable conclusions. 
The approach of death is announced by an enormous increase 
in the frequency of the pulse, by renewed convulsions, and 
deep coma. 

The chronic form begins insidiously and gradually, the first 
thing to attract our attention being the change in the disposi- 
tion of the child. Previously gay, friendly, playful, and com- 
panionable, he becomes peevish, irritable, unmanageable, and 
willful. On the least provocation he begins to cry and to be 
naughty, so that the parents find it necessary to punish him. 
It is not until the sleep begins to be disturbed and the child 
tosses about all night and groans in its sleep, wakes up in the 
morning without being rested, and complains of headache, that 
the parents become apprehensive, and the loss of appetite, the 
occasional vomiting, the obstinate constipation, and the pale, 
sickly appearance confirm the fear that a serious malady is on 
the point of showing itself. The symptoms may for weeks 
remain obscure ; high temperature may alternate with low, a 
frequent with a slow pulse, without it being possible to say 
anything definite about the case. Only when one day an epi- 
leptiform attack occurs, the headache increases in intensity, the 
child becomes somnolent, cries out during sleep, shrinks on 



1 6 DISEASES OF THE MENINGES OF THE BRAIN. 

being touched (hyperesthesia of the skin), only then is the 
condition clearer, and finally can not be mistaken when such a 
focal symptom as paralysis of the eye muscles appears. Even 
then remissions may occur, and decided improvement or even 
complete recovery is not impossible. The outlook is always 
doubtful, and can, even when the prospects appear most favor- 
able, be very serious. 

(b) In adults the difference between the chronic and acute 
form is less marked than in children. Patients who have by 
no means presented definite signs of tuberculosis begin to com- 
plain of vague headache, general prostration and malaise; their 
sleep becomes disturbed and restless ; especially in the morn- 
ing they feel tired and unstrung; they complain of loss of ap- 
petite, and may have occasional vomiting spells. In some cases 
the psychical symptoms are the most prominent, and it may 
happen that the disease begins with the symptoms of a deliri- 
um tremens, especially if the patient be a drinker. In all cases 
the sensorium becomes sooner or later dull ; the patient appears 
dazed, gives confused answers, and conveys in general the im- 
pression of a man whose mind is affected. Not rarely delirium 
comes on ; in it the excitement and exaltation are the most 
prominent features. But with all these symptoms the influence 
of a severe, agonizing headache still makes itself known, and 
even during unconsciousness the patients often raise the hand 
toward the head, throw themselves about in bed restlessly 
with groans, and seem sensitive to the slightest touch or tap on 
the head. Epileptiform seizures have repeatedly been observed 
(Meloir, Etude sur la forme epileptique de la meningite tuberc, 
These de Paris, 1888). The participation of certain cranial 
nerves, especially the oculo-motor and the abducens, is evident 
from the transient ptosis, the inequality of the pupils, and the 
strabismus ; the ophthalmoscopic examination not uncommonly 
reveals choked disk. If in looking for the latter we are able 
to find tubercles in the choroid, this is of course of the highest 
importance for the diagnosis. The facial nerve, which often 
becomes affected, may be the seat of spasm or of paresis. If 
we remember that the base of the brain is the chief seat of the 
inflammation we can easily understand why these cranial nerves 
should be implicated. If motor disturbances, consisting of gen- 
eral or unilateral convulsions, or of hemiplegia or paresis, as 
well as speech disturbances, make their appearance, we may 
assume that an eruption of tubercles has occurred in the brain 



LEPTOMENINGITIS. Y y 

cortex, an assumption which is to a certain extent supported 
by the occasional appearance of trismus. The more pronounced 
these disturbances, which are to be regarded as focal symptoms, 
the more likely is it that circumscribed tuberculous softenings 
exist in the cortex. Sometimes also a peculiar tonic rigidity 
develops in all four extremities which seems to be of reflex 
origin. The reflexes, at first increased, but presenting nothing 
characteristic, usually lose in intensity as the disease goes on, 
and finally disappear altogether. With regard to the sensory 
changes, it should be remarked that hyperesthesia of the skin 
is not so regular a symptom in this as in the first described 
form of meningitis. The temperature, as a rule, is somewhat 
above the normal, yet it varies, and occasional remissions may 
be followed by elevations, or it may remain constantly between 
ioi° and 102° F., or thereabouts. Nothing certain, however, 
can be said about it. Strlimpell reports a temperature of 88° 
during the agony. Equally variable is the pulse, which as a 
rule is slowed. We may count 40 to 50 beats a minute, while 
in a few hours it may rise to 100 or 120. 

Other organs take but a small share in the disease, and even 
the lungs show signs only when simultaneously affected with 
miliary tuberculosis. If the respiration assumes a Cheyne- 
Stokes type (after a series of shallow respirations, which be- 
come deeper and deeper, a complete pause), this is usually a 
bad omen. 

To say anything positive about the course of tuberculous 
meningitis in the adult is impossible. It is not constant, but 
sometimes acute, sometimes chronic, sometimes presenting long 
intermissions, and sometimes steadily progressive. A subdi- 
vision into different stages may look very well on paper, but to 
demonstrate them at the bedside is only rarely possible. A 
period of cerebral irritation has been distinguished from one of 
increased intracranial pressure, and this again from a period of 
paralysis. The first has been thought to be characterized by 
headache, vomiting, and delirium ; the second, by slowing of 
the pulse and paralyses ; the third, finally, by increase in the 
frequency of the pulse, elevation of temperature, and deep 
coma. But such a division entails no practical benefit, as the 
so-called " stages " are often not distinguishable from each 
other, but pass directly one into the other. From the instruct- 
ive treatise of Hirschberg (cf. lit.) we learn that even the man- 
ner of onset may vary much, and that it may be difficult even 



1 8 DISEASES OF THE MENINGES OF THE BRAIN. 

in the stage of focal symptoms to make a diagnosis. If a con- 
sumptive suddenly develops symptoms of motor or sensory 
paralysis or irritation, this should always make us suspect the 
existence of a tuberculous process in the brain. 

Diagnosis. — None of these different forms of meningitis that 
we have described is easy to diagnosticate, with the exception, 
perhaps, of the epidemic cerebro-spinal. When several cases 
have occurred in a community the recognition of new ones pre- 
sents no difficulty, especially if we keep in mind the frequency 
with which herpes labialis is met with in the disease. 

A serous meningitis may be not infrequently confounded 
with the purulent form, a fact to which Quincke has lately 
called attention in his excellent paper (Sammlung klin. Vortr., 
N. F., Leipzig, 1893, No. 67). The absence, or the slight de- 
gree, of fever, often also its irregular appearance, together with 
the relative mildness of the manifestations pointing to cortical 
involvement, such as headache, stiffness of the neck, and 
clouding of consciousness, and on the other hand the relative 
frequency of choked disk, are the features which are more 
characteristic of the serous form. 

Of other diseases, typhoid fever is perhaps the most likely 
to be mistaken for meningitis. There is no doubt, and it has 
been shown by reliable observers (Curschmann), that there are 
cases in which meningitic symptoms are very well marked, but 
in which typhoid bacilli are found in the cord at the autopsy 
to be the infective agent. We might be led to believe that at 
least the characteristic temperature curve, the splenic enlarge- 
ment, the condition of the stools, and the rose spots would be 
sufficient to make a mistake impossible, but this is by no means 
always the case ; there are instances in which typhoid fever 
can not with certainty be excluded, and then the differential 
diagnosis is simply impossible. 

If ursemia enters into the question of diagnosis, the exami- 
nation of the urine (for tube-casts, etc.), suppression of the urine, 
if it should be present, and the appearance of the convulsions 
will facilitate the recognition of the true condition. 

Whether we have to do with a case of croupous pneumonia 
or with meningitis is, in the majority of cases, easy enough to 
decide. Both affections may, however, occur together, and 
then it is important to remember that marked hyperesthesia 
of the skin, staggering gait, and rigidity of the neck may all be 
present with pneumonia alone. If this be complicated by 



LEP TOMENINGITIS. ! q 

oedema of the glottis, so that respiration is difficult, the patient 
will fix his head in order to bring- into play the auxiliary mus- 
cles of respiration, and thus in the recumbent position too the 
rigidity of the neck is simulated (Wernicke). The existence 
of meningitis is only, then, to be assumed if pronounced basal 
symptoms are present, and especially if paralysis of the eye 
muscles has existed for a certain period of time. 

More frequently delirium tremens is associated with men- 
ingitis, and we are not always able to decide whether the 
delirium, the tremor, and the epileptiform convulsions are 
referable to the latter or to the former. 

It is well to remember that there are cases in which, al- 
though the symptoms of tuberculous meningitis seem pro- 
nounced, in a few weeks the patient completely recovers, in 
which instances the assumption that there is a pseudo-men- 
ingitis of hysterical origin seems necessary (Carrier, Lyon 
med., October, 1892, lxxi). Of course, the previous history of 
the patient, the family history, etc., have to be taken into con- 
sideration before such a diagnosis, which we think is always 
very risky, can be even thought of. Of interest are the ob- 
servations of Carin and Iscovesco (La France med., 136, 1888) 
upon a diagnosis of meningitis in cases of iodoform poisoning. 

The occurrence of meningitic symptoms as a consequence 
of worms, which Devaux (cf. lit.) has upheld, is certainly ex- 
ceptional, and can hardly, for any length of time, give rise to 
an error in diagnosis. 

With sufficient care we can easily avoid confounding men- 
ingitis with eclampsia infantum. 

Prognosis. — The prognosis in every case of meningitis is 
very serious ; we are never in a position to predict with any 
certainty the outcome, not even when everything seems to be 
going on very favorably, and grave symptoms have not de- 
clared themselves. These may suddenly develop in one night, 
and a patient whom we have left in fairly good condition in 
the evening may the following morning be hopelessly ill. On 
the other hand, we should not give up our patient too soon ; the 
gravest symptoms may fade away, and improvement is still 
possible even where the case seems desperate. Undoubtedly, 
however, meningitis is one of the most serious diseases, and 
one in which recovery is rare, the epidemic cerebro-spinal 
meningitis being the only form which sometimes runs a more 
favorable course. 



20 DISEASES OF THE MENINGES OF THE BRAIN. 

Partial recoveries are much more often seen than absolute 
ones. If, for example, in the course of meningitis, a hsemor- 
rhagic inflammation of the inner ear develops, this gives rise to 
permanent deafness, which in younger children, as a rule, leads 
to deaf-mutism (Schulze, Taubstummheit und Meningitis, 
Virch. Arch., 1890, cxix, p. 1), or if purulent inflammation of 
the eyeball, a panophthalmitis or a choroiditis coexist with the 
meningitis, this may entail a grave disturbance of sight, even 
phthisis bulbi, and complete amaurosis. In either of these 
cases the meningitis may get well, but leave in one deafness, in 
the other impairment or loss of sight, and in the most unfavor- 
able cases both remain behind without the development of any 
mental defects. Blindness may also be a consequence of an 
optic neuritis, which does not get well, but causes shrinking of 
the optic nerve and atrophy of the disk. Cases of meningitis 
confined to the convexity sometimes recover, leaving a more 
or less marked feeble-mindedness. 

Treatment. — The treatment is first to be directed against 
the inflammation, and later endeavors should be made to aid 
absorption of exudates if such be present. For this purpose 
we make use of so-called surgical revulsives (Erlenmeyer, 
Deutsche med. Ztg., 1893, p. 61); for example, local bleeding 
and the application of cold inunctions of mercurial ointment, 
four to eight grammes (3j to 3 ij) a day to the shaved head, 
or blisters (Mosler, Deutsche med. Wochenschrift, 1888, No. 
30, p. 621). In some cases we shall succeed with such meas- 
ures in lessening the severity of the symptoms, but often little 
or nothing is achieved by them. Painting the shaved head 
with tincture of iodine is objectionable, owing to the disagree- 
able and painful tension which it produces, and which is but 
little alleviated by ice. That free purgation with large doses 
of calomel actually produces an antiphlogistic effect can not be 
proved, but there is no reason why it should not be tried, the 
drug being given until the characteristic stools appear. The 
absorption of exudates is attempted by large doses of potas- 
sium iodide, four to six grammes (3j to 3jss.) a day in hot 
milk, a medication which is especially indicated in the gumma- 
tous form of meningitis. 

During coma the patient may be put into a tepid bath 
(90 to 93 F.) and cold water (66° to 6o° F.) be poured over 
his head. These cold-water affusions may be continued for 
eight or ten minutes, with the frequent result of actually rous- 



LEPTOMENINGITIS. 21 

ing the patient out of his unconsciousness, an improvement, 
however, which generally does not last very long. The repe- 
tition of this procedure several times a day is therefore neces- 
sary, notwithstanding the considerable difficulties with which 
it is (at least in private practice) attended. 

Symptomatically the agonizing headache and the jactita- 
tions may be met with morphine. The same drug is used 
against the obstinate vomiting, which is hard to treat, and in- 
deed may resist all efforts. It may happen that all internal 
medicines, cracked ice, champagne, opium, aromatic tinctures, 
etc., as well as all applications of spiritus sinapis, etc., remain 
without effect ; then we are forced to resort to morphine, the 
subcutaneous administration of which generally accomplishes 
more than all remedies previously used. The regulation of 
the bowels should of course never be overlooked. 

We can only, then, with reason hope for success from our 
therapeutic efforts if we pay careful attention to the nutrition 
of the patient. As soon as this is left out of sight the battle 
is practically lost in spite of all medicines and inunctions. 
More than in any other disease it is here the chief task of the 
physician to see that the strength of his patient is kept up, so 
that he be fit, if necessary, to stand an illness of weeks ; and 
more than in any other disease is here the prolonged use of 
wine indicated, and is much more important than all drugs. 
Besides wine, a tablespoonful of beef-tea is to be given every 
hour. This is prepared by gradually heating lean beef cut into 
small cubes, after the addition of a little salt, in a lightly closed 
glass bottle over the water-bath, and cooking it until the pieces 
are completely disintegrated. Two pounds of meat furnish 
about a cupful of beef-tea. 

In very exceptional cases operative measures are indicated, 
namely, where we have sufficient reason to suspect the exist- 
ence of an exudate in the ventricles, which would manifest 
itself by an aggravation of the symptoms of increased intra- 
cranial pressure. Trephining and tapping of the ventricles 
(Keen, Philadelphia) may then be resorted to if the circum- 
stances are in other respects favorable. In cases of otitis media 
the tympanic membrane should be punctured and the cavity 
syringed out with antiseptic solutions. It is scarcely to be 
expected that the treatment of tubercular meningitis by para- 
centesis of the spinal canal, a procedure practiced in four cases 



22 DISEASES OF THE MENINGES OF THE BRAIN. 

by W. Essex Wynter (Lancet, May 2, 1891), will meet with 
general acceptance. 

The treatment of tuberculous meningitis in children has to 
be conducted according to the plans just laid down, with this 
difference, that the inunctions of the head with mercurial oint- 
ment are to be replaced by the administration of calomel, 
three to five centigrammes (grs. ss. to j) every two hours. 
Besides, the inunctions of the head with iodoform ointment, 
lately so warmly recommended, should be tried ; but here, too, 
the preservation of the strength must be our chief aim. Milk, 
with the addition of a little Hungarian wine or a few drops of 
cognac, should always be kept ready. 

LITERATURE. 

Bull. Ueber die Kernig'sche Flexionscontractur der Kniegelenke bei Gehirn- 

krankheiten. Berl. klin. Wochenschr., 47, 1885. 
Leyden. Bermerkungen iiber Cerebrospinalmeningitis und iiber das Erbrechen 

in fieberhaften Krankheiten. Zeitschr. f. klin. Med., xii, 4, 1887. 
Devaux. Oxyures et symptomes pseudo-meningitiques. Progr. med., No. 46, 

1887. 
J. Simon. Diagnostic differentiel de la meningite tuberculeuse. Gaz. des Hop., 

No. 132. Nov., 1887. 
Wolff, Felix. Bemerkungen iiber das Verhalten der Cerebrospinalmeningitis zu 

den Infectionskrankheiten. Deutsche med. Wochenschr., 50, p. 1080, 

1887. 
Weichselbaum. Ueber die Aetiologie der acuten Mening. cerebro-spin, Fort- 

schr. d. Med., 18, 19, 1877. (" Diplococcus intercellularis meningitidis.") 
Hofmann v. Ueber die acute Meningitis in angeblich ursachlichem Zusammen- 

hange mit Misshandlungen oder leichten Verletzungen. Wiener med. 

Wochenschr., 6, 1888. 
Pio Foa und Guido Bordoni-Uffreduzzi. Ueber die Aetiologie der Meningitis 

cerebrospinalis epidemica. Zeitschr. f. Hygiene, 1888, iv, No. 1, pp. 67 

et seq. 
Baaz. Die Cerebrospinalmeningitis, ihr Wesen und ihre Behandlung. Berlin- 

Neuwied, Heuser, 1888. 
Freyhan. Zur Kenntniss der Typhusmeningitis. Deutsche med. Wochenschr., 

1888, No. 31, p. 630. 
Wolff, Felix. Ueber meningitische Erscheinungen beim Typhus abdominalis. 

Ziemssen's und Zenker's Archiv, 1888, xliii, Heft 2 u. 3, p. 250. 
Stephan. Des Paralysies pneumoniques. Revue de med., 1889, ix, No. 1. 

(" Meningitis as a Complication of Pneumonia.") 
Adenot. Des meningites mikrobiennes. Paris, Bailliere, 1890. 
Fox. Amer. Journ. of the Med. Sciences, June 6, 1890, xcix. 
Oebeke. Ueber Meningitis cerebrospinalis. Berliner klin. Wochenschr., 1891, 

No. 41. 
Hilbert. Berliner klin. Wochenschr., 1891, No. 31. 



LEP TOMENINGI TIS. 2 3 

Matthes. Linksseitige Hypoglossuslahmung bei tuberculoser Meningitis. 

Miinchener med. Wochenschr., 1892, No. 49. 
Trevelyan. Cerebro-spinal Meningitis. Brain, Spring Number, 1892. 
Schwabach. Ueber Gehbrstorungen bei Meningitis cerebrospinalis und ihre 

anatomische Begriindung. Zeitschr. f. klin. Med., 1892, xviii, No. 3 u. 4, pp. 

273-297. 
Mertz. Deutsche med. Wochenschr., 1892, xix, No. 2. 
Allya. Three Fatal Cases of Cerebro-spinal Meningitis, with Autopsies. Med. 

News, May 14, 1892. 
Maulwurf. Wiener med. Wochenschr., 1892, xlii, No. 47. 
Mensi e Carbone. Riforma med., 1893, ix, No. 2. 
Zbrkendbrfer. Zur Bacteriologie der Meningitis suppurativa. Prager med. 

Wochenschr., 1893, No. 18. 
Boix. Revue de med., 1893, p. 413. 
Randolph. Bull, of the Johns Hopkins Hospital, July 4, 1893 (forty cases of 

meningitis, examined clinically). 
Klemperer. Ueber die Bedeutung des Herpes labialis bei der Cerebrospinal- 

meningitis. Berliner klin. Wochenschr., 1893, No. 29. 
Friis. Ugeskr. f. Laegenidensk, 1893, xxvi, No. 27-29. (" On Meningitis 

cerebrospinalis epidemica.") 



PART II. 

DISEASES OE THE CRANIAL NERVES. 

If we once have a clear idea that in the cranial nerves we 
have to distinguish the origin, which in all probability is found 
in the cortex and the nuclear region of the medulla oblongata, 
from the partly central (intracerebral), partly peripheral (ex- 
tracerebral) course, it is self-evident that the diseases of the 
cranial nerves are divisible into those which affect the nerve at 
its origin, the center, and those which affect it in its course. As 
we shall come to deal in the next part of our book with the 
affections of the brain substance proper, it necessarily results 
that in the following chapters we must either touch upon 
things which properly belong to Part III, or that in the latter 
we shall not be able to avoid some repetition. Neither of these 
courses is without objections ; still, from a practical point of 
view, we have deemed it best to treat of the diseases of the 
cranial nerves here in toto. 

The central lesions of the cranial nerves often form merely 
a part of a more general disease of the nervous system. Those 
of peripheral origin occur also independently — for instance, as 
the result of exposure to cold, traumatism, etc. In very many 
cases we are not able to determine definitely whether the dis- 
ease has a central or a peripheral origin. For a clear under- 
standing of the following chapters, a knowledge of the anatomy 
of the parts naturally can not be dispensed with. Some remarks 
bearing on this, which, of course, are not meant to take the 
place of a detailed study, have therefore been inserted at the 
head of each chapter to recall to the reader's mind in outline 
the necessary anatomical relations. 
24 



DISEASES OF THE OLFACTORY NERVE. 2 $ 

CHAPTER I. 

DISEASES OF THE OLFACTORY NERVE. 

The olfactory nerve begins in a small pyramidal lobule, the tu- 
ber olfactorium (caruncula mamillaris), the base of which is situated 
in front of the anterior perforated space. At its beginning, the nerve 
is broad, but narrows into a band somewhat prismoidal on section, 
which is called the olfactory tract, and which in its turn ends in an 
oval gray swelling, the olfactory bulb. From the lower aspect of 
this bulb, which lies on the cribriform plate of the ethmoid bone, two 
groups of fibers pass through the little openings of the bone into the 
nasal cavity, and it is only the sum of these filaments (the fila olfac- 
toria) which can be looked upon as the nerve of smell in the strict 
sense of the term. The olfactory tract and bulb are parts of a cere- 
bral lobe, the so-called olfactory lobe. 

The origin and the course of the roots of the olfactory nerve (the 
striae olfactorii, Schwalbe) are not known. It is, however, generally 
agreed that there are three roots. The outermost, the strongest, is 
said to be traceable into the island of Reil. Schwalbe supposed the 
existence of a lateral root (radix lateralis, seu longa, seu externa) 
originating in the hippocampal convolution, and of a median (radix 
medialis, seu interna, seu brevis), coming from the gyrus fornicatus. 
Others have looked upon the anterior commissure and the corpus 
striatum as the starting points of the olfactory nerve, but nothing 
positive is known. An olfactory center has been assumed in the 
gyrus hippocampi and in the gyrus uncinatus. Lately Zuckerkandl 
(cf. lit.) has claimed that the cornu Ammonis is a part of the olfactory 
center (cf. also the extensive paper by Troland, De l'appareil nerveux 
central de l'olfaction, Arch, de Neurol., 1891, lx, p. 335 ; lxii, p. 183; 
lxiv, p. 69 ; lxv, p. 203). 

Notwithstanding the fact that the affections of this nerve 
are not of very great practical importance, they afford a great 
deal of interest, because they may under certain circumstances 
(i. e., if a careful clinical description is followed by an exact 
and accurate post-mortem account) give us some information 
about the anatomical and physiological questions concerning 
the course and origin of the nerve, and again because they may 
attain a considerable importance and value in the diagnosis of 
certain cerebral diseases. 

The olfactory nerve may be diseased in its central or in its 
peripheral portion. In the former case it may be the olfactory 



2 6 DISEASES OF THE CRANIAL NERVES. 

center which is affected, or the conduction may be interfered 
with somewhere in the course of the intracerebral paths. 

Since, as we have stated, the situation of the olfactory cen- 
ter is not definitely known, we can not be expected to know 
much about its diseases. It would appear, however, that it may 
be affected by destructive as well as by irritative lesions ; the 
latter manifest themselves by hallucinations, the former by loss 
of smell (anosmia). Among the diseases in which hallucina- 
tions of smell occur are various psychoses, also migraine, tic 
douloureux, epilepsy, and tabes. Usually the smell which such 
patients describe is bad, disgusting — of faeces, sometimes of 
poisonous plants, putrid substances, etc. (kakosmia) — and it is 
rare for them to imagine that they smell pleasant substances. 
One of my cases, who, owing to an ocular paralysis, was treated 
with the galvanic current passed through his head from one 
side to the other, declared that he smelled oil of lavender from 
the moment the current was closed until it was again broken. 
This seems to point to the possibility that by the galvanic cur- 
rent the olfactory center may be stimulated. Central anosmia 
is sometimes observed in cerebral lesions following fracture of 
the skull, which cause hemiplegia and aphasia, the disturbance 
being confined to the nostril on the same side as the lesion. 
Anosmia is also known to occur in hysteria and in old age ; in 
the latter case it is probably to be attributed to atrophy (senile 
anosmia). Cases have been repeatedly noted in which tumors 
of the anterior fossa of the skull, exostoses, meningitis at the 
base of the frontal lobe, have given rise to anosmia. The fact 
that several odors acting on the olfactory nerve at the same 
time suspend each other is probably to be explained on physio- 
logical grounds, as is also the fact that the acuteness of olfac- 
tory perception is diminished if at the same time another 
cranial nerve — e. g., the optic or the auditory — is strongly 
stimulated. 

Interference with conduction in the olfactory nerve may 
be assumed in cases where there is a history of traumatism — a 
fall upon the head, more especially upon the occiput. Accord- 
ing to Carbonieri, complete loss of smell suggests strongly dis- 
ease of the olfactory tract or bulb. 

The treatment in the central affections of the olfactory 
nerve must of course be directed against the underlying 
disease. 

Of greater practical interest are the peripheral affections of 



DISEASES OF THE OLFACTORY NERVE. 2 7 

the olfactory, which chiefly consist in a decrease of the power 
of smell. Leaving out of consideration the common cases in 
which an acute or chronic nasal catarrh causes partial or, tem- 
porarily, even complete anosmia, the sense of smell may be 
affected as the result of abnormal dryness of the nasal cavity 
(diminution in the secretion of tears in trigeminal anaesthesia, 
diminished flow of tears into the nasal cavities in facial paral- 
ysis). Not rarely certain occupations give rise to anosmia, 
which is sometimes associated with a tolerance of disagreeable 
odors which at first were highly obnoxious to the workers. 
Such anosmias are to be found in soap-boilers, catgut spinners, 
tanners, skinners, and butchers, whose sense of smell is often 
considerably dulled ; again it may be due to disturbances in 
nutrition, to the action of caustic substances, or injury to the 
peripheral nerve endings — effects which are due to the chemi- 
cal composition of the inhaled substances. Thus we have ob- 
served loss of the sense of smell in those working in chlorinated 
lime, while it was found to be diminished in laborers occupied 
with the pulverization of chrome-ironstone. Strieker has also 
known it to occur in an entomologist in consequence of the 
protracted inhalation of ether. 

I have observed hyperesthesia of the olfactory nerve in 
hysterical women especially during pregnancy, and also during 
galvanization of the brain (v. s.). 

The treatment consists in faradization (Beard and Rockwell) 
and galvanization (Fieber) of the nasal cavity, or painting with 
a one-per-cent solution of strychnine (in olive oil). The use of 
irritative snuff powders has repeatedly been recommended for 
anosmia of peripheral origin, but has frequently been used 
without benefit. Spontaneous recovery is not rare. Finally, 
we may say a word or two about the method of testing the 
sense of smell. All those substances which irritate the trigem- 
inus should be avoided, as, for instance, acetic acid, smelling 
salts, snuff, tobacco ; the patient would feel what he can not 
smell, and we might be thus led astray in our conclusions. 
Cologne water, oil of rosemary, musk, camphor, anise, oil of 
turpentine, asafcetida, and sulphureted hydrogen, are sufficient 
for most tests. That each nasal cavity must be tested sepa- 
rately goes without saying. A special olfactometer has been 
devised by Zwaardemaker (Berl. klin. Wochenschr., 1888, No. 
47; Fortschritte der Med., 1889, No. 19), and another more 
recently by Savelieff (Neurol. Centralblatt, 1893, No. 10). 



28 DISEASES OF THE CRANIAL NERVES. 



LITERATURE. 

Notta. Recherches sur la perte del'odorat. Arch, gener. de med., April, 1870. 
Ogle. Anosmia, or Cases illustrating the Physiology and Pathology of the Sense 

of Smell. Med.-chir. Transact., 1870, liii. 
Molliere. Note pour servir al'histoire du nerf olfactif. Lyon med., 1871, No. 20. 
Carbonieri. Zur Localisation des Centrum olfactorium. Riv. clin., xxiv, 9, p. 

657, September, 1885. 
Erben. Wien. med. Blatter, 1886, No. 43, 44 (kakosmia in tabes). 
Moldenhauer. Die Krankheiten der Nasenhohlen u. s. w. Leipzig, Vogel, 1886. 
Gowers. Lectures on the Diagnosis of the Diseases of the Brain, delivered at 

University College Hospital, London. Churchill, 1885. Also Blakiston, 

Philadelphia, 1885. 
Thudichum. On the Nature and Treatment of Hypertrophies and Tumors of 

the Nasal and Pharyngeal Cavities. The Lancet, August 27, 1887, p. 401. 
Zuckerkandl. Ueber das Riechcentrum. Stuttgart, Enke, 1887. 
Roth. Die Erkrankungen der Nasenschleimhaut, ihre Beziehungen zum iibrigen 

Organismus und Behandlung derselben. Centralbl. fur d. ges. Therapie, v, 

Heft x, October, 1887. 
Zwaardemaker. Berliner Klinik. Reviewed in Wiener med. Presse, 1890, 

No. 39. 
Zwaardemaker. Zur Methodik der klin. Olfactometrie. Neurol. Centralblatt, 

1893, No. 21. 



CHAPTER II. 

DISEASES OF THE OPTIC NERVE. 



The optic nerves derive their fibers from the occipital lobes, the 
optic thalami, the outer and inner geniculate bodies, the anterior 
corpora quadrigemina, and the cerebellum (through the superior 
peduncle of the cerebellum). 

What are known as the optic tracts before the chiasm is reached, 
after this point become the optic nerves. These are round hard cords, 
about four millimetres in diameter, which, passing in a diverging 
direction through the optic foramina, enter the orbits and reach the 
eyeballs after their passage through the orbital fat. Here they pass 
the sclerotic and choroid and spread themselves over the fibrous 

layer of the retina. The outer 
covering of the nerve, which is 
a process of the dura mater, is 
called the dural sheath ; the 
process of the pia, the inner 
or pial sheath. The two are 
separated by a space which be- 
longs to the lymphatic system, 
the so-called intervaginal or 
subvaginal space. The arteria 
centralis retinae, a branch of 
the internal carotid, enters the 
optic nerve about fifteen or 
twenty millimetres from the 
eyeball and runs together with 
the vein of the same name in 
the substance of the nerve to the retina. 

The chiasm, which is formed by the union of the optic tracts, is a 
flattened four-sided body, in which the crossing of the optic fibers 
takes place. This crossing, as we now know with a fair amount 
of certainty, is, however, only partial, a semidecussation. The fibers 
from the outer half of the retina (represented by an interrupted line) 
pass to the center without decussating, while those of the inner half 

29 




Tract 



Fig 



2. — Diagram showing the Course of 
the Optic Fibres in the Chiasm. 



30 



DISEASES OF THE CRANIAL NERVES. 



cross over and pass to the centre of the opposite side (cf. Fig. 2). 
Each occipital lobe, therefore, receives fibres coming from the tem- 
poral as well as from the nasal half of the retina. Thus, for in- 
stance, the left receives fibres from the outer temporal half of the 
left and from the inner nasal half of the right retina. In diseases of 
this lobe, therefore, images falling upon the left half of the retina, 
or, in other words, those which lie in the right half of the field of 
vision, are no longer perceived — right-sided hemianopia. 

The optic tract, the superficial fibres of which can be traced into 
the white covering of the pulvinar (the so-called stratum zonale thai- 
ami), originates by two roots — an outer, much stouter, the end ganglia 
of which are the anterior corpus quadngeminum, the outer genicu- 
late body, and the pulvinar, and by an inner root which can be 
easily followed to the inner geniculate body (Wernicke). These end 
ganglia of the optic tracts form at the same time the terminal points 
of certain fibres of the corona radiata, which run in a sagittal direc- 
tion forward from the occipital lobe, and are connected with the 
pulvinar, the brachium anterius of the quadrigeminal body, and the 
outer geniculate body. This bundle of fibres is the sagittal medul- 
lary tract of the occipital lobe, or what is called the optic radiation, 
and is designated in the diagram by s {vide Fig. 3). 

The exact localization of the cortical centre of vision has not as 
yet been established. According to Ferrier, it is in the angular 
gyrus; according to Munk, it is in the convex surface of the occip- 
ital lobe. 

It would be beyond the scope of the present work to treat 
in extenso of those diseases of the optic nerve which belong- 
strictly to the domain of ophthalmology ; they can be con- 
sidered here only so far as they are connected with the nervous 
system. To these belong, first of all, certain inflammatory con- 
ditions which act upon the intraocular end of the nerve, the 
papilla (disk), and give rise to what we therefore term papil- 
litis (choked disk). The name optic neuritis, which is fre- 
quently used as a synonym for papillitis, is inexact, because it 
may imply an affection of the whole nerve trunk. 

The papillitis, choked disk {Stauungspapille, as the Germans 
call it, after von Graefe, 1859), * s frequently, although not al- 
ways, met with in cases of intracranial tumors, and is (accord- 
ing to von Graefe) to be attributed to a high grade of venous 
engorgement, produced by an impediment to the reflux of the 
venous blood into the skull cavity. Later, when Schwalbe had 
discovered that there was a communication between the fluid 



DISEASES OF THE OPTIC NERVE. 



31 



contents of the skull and the intervaginal space of the optic 
nerve, it was shown that the subdural space was distended 
with a serous inflammatory fluid, and that the optic nerve at its 
passage through the lamina cribrosa of the sclerotic becomes 
compressed (Schmidt-Rimpler). Finally, Deutschmann (cf. lit.) 




\ 



Fig. 3. — Diagram showing the Origin of the Optic Nerve. (After Wernicke.) 
p, crusta of the crus cerebri ; sn, substantia nigra ; cgi, inner, cge, outer geniculate 
body ; qp, brach. post. corp. quadr. ; qa, brach. ant. corp. quad. ; pit, pulvinar ; s, optic 
radiation. 

has put forth the view that papillitis is not caused by mechan- 
ical influences, but that it is due to the action of pathogenic 
organisms which enter from outside. How far this view is 
correct further experience will show. Besides the pure papil- 
litis there is also found a papillo-retinitis, the ophthalmoscopic 
picture of which differs from that of the former affection, and 
which is to be referred to a meningitis, which has advanced 
along the sheath of the optic nerve. 

A pure papillitis, as we have said, is chiefly found in intra- 
cranial tumors. Patients in whom a brain tumor is suspected 
ought to be examined for choked disk even if they do not 
complain of any subjective symptoms pointing to it, because 
sight may, even if the disk is markedly swollen, remain nor- 
mal for a long time. Only when the nerve or the chiasm is 
strongly compressed does amblyopia or amaurosis occur in 
the early stages. 



32 DISEASES OF THE CRANIAL NERVES. 

The seat of the tumor has nothing to do with the occur- 
rence of papillitis. Basal neoplasms can, through direct press- 
ure upon the optic nerve, cause a simple atrophy of the same. 
Nor does the nature of the tumor play any part here. Gum- 
mata, tubercles, entozoa (cysticerci, echinococci), carcinomata, 
gliomata — any one of these may produce a papillitis, which is 
usually bilateral (in ninety-three per cent of the cases, Annuske 
and Reich), although the processes need not necessarily be 
equally developed in both eyes. 

Of practical importance are the sudden spells of blindness 
which occur sometimes in the course of a papillitis, termed by 
H. Jackson epileptiform amaurosis. They are probably due 
to a temporary swelling of certain tumors and the consequent 
compression of certain areas of the brain or the vessels (Leber) 
distributed to them. These attacks may last for hours or days, 
and either disappear completely or leave a permanent increase 
in the amblyopia The ophthalmoscopic examination does not 
teach us anything about this periodical blindness. 

A papillitis rarely ever gets well ; in by far the greater 
number of cases a papillitic atrophy and total amaurosis take 
place, first in one and then in the other eye. Cases in which 
one eye is seriously damaged while the other remains per- 
fectly well are extremely rare. I have, however, had occasion 
to observe an instance of this with Magnus. More frequently 
both eyes become diseased, one soon after the other. Dropsy 
of the ventricles may give rise to a simultaneous amaurosis of 
both eyes. 

Papillo-retinitis is not very rare in tubercular basilar men- 
ingitis ; in epidemic cerebro-spinal meningitis it is exceptional. 
Chronic cerebral affections of children often lead to it, the 
amaurosis in these instances usually developing quickly, while 
the general symptoms become intensified. 

Inflammations of the optic-nerve trunk occurring alone may 
be caused by cold, febrile diseases, syphilis, disturbances in 
menstruation, and hereditary influences. On ophthalmoscopic 
examination either nothing remarkable or only a slight blurring 
of the disk is recognizable, because the inflammation affects 
more especially that part of the nerve which is behind the eye- 
ball (retrobulbar neuritis of von Graefe). The disturbance of 
vision usually begins gradually, and is confined either to the 
periphery of the field of vision or it consists of a central am- 
blyopia or a circumscribed central amaurosis. It does not ter- 



DISEASES OF THE OPTIC NERVE. 



33 



rainate in complete blindness ; frequently only marked disturb- 
ance of color vision remains. 

To the neurologist the cases of optic neuritis in patients 
with a neuropathic family history are of extreme interest. 
Such persons usually suffer even in early youth from migraine, 
nervous palpitation of the heart, vertigo, sometimes also from 
epileptiform attacks. Between the ages of twenty and thirty 
they begin to complain of trouble with their sight, either of 
subjective light or color sensations or else that objects appear 
to them enveloped in a dense mist ; within from four to six 
weeks they may become completely blind, but their blindness 
as a rule does not persist, but gives place to a central ambly- 
opia with normal sight at the periphery of the field of vision. 
The prognosis differs markedly in different families. It is of 
interest to note that as a rule only the male members of the 
family are wont to be affected by the disease. 

In the second place we will consider atrophy of the optic 
nerve. It consists in a wasting of the nerve elements, and may 
be either primary (genuine) or inflammatory, the consequence 
of a previous neuritis. It may also affect the trunk of the 
nerve as well as the intraocular end of it. If the nerve, besides 
the wasting of its pulp, also undergoes a diminution in its 
volume, so that it appears like a gelatinous grayish-yellow cord, 
the atrophy is known as gray degeneration. 

Tumors and inflammatory exudates, as well as splinters of 
bone, may by pressure, by shutting off the blood supply (as, 
for instance, in embolism of the arteria centralis retinae), and 
through interference with the nutrition lead to atrophy. 

The progressive atrophy, or, as it is better termed, progress- 
ive gray degeneration, which may be of cerebral or spinal 
origin, is characterized clinically by a diminution in the acute- 
ness of the central vision, a contraction of the whole visual 
field, and disturbance of the color sense. In the ophthalmo- 
scopic examination the bluish-white discoloration of the disk 
and the atrophic excavation of the nerve (due to wasting of the 
substance of the disk) are very apparent. The acuteness of 
vision, grows gradually but progressively less, and months and 
years may pass before complete amaurosis is developed. On 
the other hand, the whole process may run its course in two or 
three weeks. The contraction of the field of vision is rarely 
concentric ; usually the defects are in one direction only, and are 
often sectorial (Leber). Enormous contraction of both fields of 
3 



34 



DISEASES OF THE CRANIAL NERVES. 



vision, with at the same time normal acuteness of sight in the 
center, which was eventually followed by blindness, has been 
observed by Schweigger. The disturbance in color vision is at 
first limited to the perception of green, which is confused with 
white or gray, the perception of blue and yellow being rela- 
tively longest retained. The atrophy develops bilaterally, al- 
though one eye alone may at first be affected, and the other 
eye remain intact for years. 

Foci of softening in the brain, progressive paralysis of the 
insane, sometimes also epilepsy, are the cerebral diseases in 
which the affection is not rarely observed. It is besides also 
noted in multiple sclerosis, although in this disease it never 
leads to total amaurosis, a fact which Charcot was in the habit 
of emphasizing in his lectures. 

More important is the fact that in locomotor ataxia optic 
atrophy is comparatively frequent. Wharton Jones (British 
Medical Journal, July 24, 1869) makes the sympathetic respon- 
sible for this, assuming that the paralysis of the vaso-motor 
nerves, producing first hyperasmia, leads finally to atrophy of 
the optic nerve. This explanation, however, is at once over- 
thrown by the fact that in the optic atrophy of tabes there are 
at no time any traces of hyperasmia. 

Congenital optic atrophy can sometimes be traced to hered- 
itary influences, or to consanguinity of the parents ; several 
cases have been known to occur in the same family without 
apparent cause (Nicolai, Nederl. WeekbL, 1890, i, 5); some- 
times it is due to hydrocephalus. Injury to the skull in conse- 
quence of instrumental interference at birth very rarely has 
anything to do with it. 

The diseases of the chiasm and optic tract may be consid- 
ered together, since they possess one symptom in common 
which is of special interest to the neurologist, viz., hemianopia. 
It is the only form of visual disturbance where one can with 
certainty diagnosticate a central affection of the optic nerve. 
It is likely to be of cortical origin if the hemianopia occurs sud- 
denly as the only symptom, there being no change to be found 
on ophthalmoscopic examination ; whereas if other symptoms 
accompany it — aphasia, hemiplegia, etc. — this idea of a cortical 
lesion must be given up. By hemianopia in general we mean 
a loss of one half (the right or the left) of the field of vision, 
so that patients affected with right-sided hemianopia see the 
objects which are in the left half of their visual field, whereas 



DISEASES OF THE OPTIC NERVE. 



35 



those to the right are not perceived. If the disturbance affects 
the halves on the same side of both eyes — that is, the nasal on 
the one, the temporal on the other — we call it a homonymous 
hemianopia. If in both fields the temporal halves are lost, this 
constitutes what is known as temporal hemianopia, which is of 
rarer occurrence ; the absence of both nasal halves of the field 
of vision does not seem to occur, and the superior and inferior 
hemianopia, where the line of division is not vertical but hori- 
zontal, seems to be extremely rare. 

The explanation of the hemianopia in lesions of the cortical 
center for sight is quite evident if we accept, as is now gener- 
ally done, the existence of the above-described semidecussation 
of the fibres in the chiasm. The path from the optic tract to 
the cortex of the occipital lobe may be divided into the follow- 
ing segments (Wernicke) : The first includes the optic radia- 
tion in the occipital lobe, the lesions of which give rise to 
homonymous hemianopia without any other focal symptoms, 
lesions of the right occipital lobe causing left-sided, those of 
the left right-sided, hemianopia ; the second will include the 
place where the fibres of the optic radiation enter the internal 
capsule, and the ganglia of origin of the optic tract, the pul- 
vinar, and the outer geniculate body — hemianopia and hemian- 
sesthesia ; the third will include the optic tract in its course at 
the base of the brain — hemianopia with hemiplegia. If in the 
region of the visual center or the optic radiation a bilateral 
focal lesion occurs, then we may have complete blindness set- 
ting in with an apoplectiform attack. This is in reality a bilat- 
eral hemianopia, and is designated cortical blindness. The 
function in the two halves of both eyes need not be totally 
lost ; atrophy of the optic nerve does not take place. Weir 
Mitchell has shown that a lesion of the chiasm may produce 
bilateral hemianopia ; his case was one in which an aneurism 
pressed upon the chiasm (Journal of Nervous and Mental Dis- 
eases, January, 1889). 

Of diagnostic value in these cases is sometimes the so-called 
hemianopic pupillary reaction (Heddaeus, Wernicke), or hemi- 
anopic inactivity of the pupil (Leyden). With the mirror of 
the ophthalmoscope we reflect the light first upon the left, then 
upon the right half of the retina, and observe the pupillary re- 
action. If the reflex occurs normally, the optic tract must be 
intact, and the disturbance must be due to a bilateral lesion of 
the optic radiation in the occipital lobe, or in the cortical cen- 



36 DISEASES OF THE CRANIAL NERVES. 

ter. If the reflex is not obtained, we must assume a lesion of 
the optic tract of the corresponding side. Light perception 
and pupillary reflex go in this case hand in hand. In a recent 
article Heddaeus himself expresses the opinion that for the 
present it is not justifiable to base the differential diagnosis be- 
tween lesions of the optic tract and lesions of the fibres in their 
central course exclusively upon the absence or presence of this 
symptom (Deutsch. med. Wochenschr., 1893, No. 3). 

In diseases of the chiasm hemianopia has been repeatedly 
met with, but in this case we have not a homonymous but a 
bitemporal hemianopia, as in the case of Oppenheim, where 
gummatous disease of the chiasm was responsible for the dis- 
turbance (cf. Virch. Arch., 1886, Bd. civ, 2, p. 306). Quite 
lately the same author has described an "oscillating" bitem- 
poral hemianopia in diseases of the chiasm, which he considers 
as pathognomonic of basal cerebral syphilis (cf. lit.). 

If the tissue injured by the lesion which has caused the 
hemianopia is capable of regeneration, as may be the case where 
we have a haemorrhage or an inflammation, the defect will pass 
off completely ; whereas if this is not the case the trouble re- 
mains stationary, without, however, any additional disturbance 
of sight. Such a condition, which often develops as the con- 
sequence of an apoplexy, may persist for years, but no second 
attack, by which the centers of the other tract also may be 
disturbed, is to be feared, as such a thing has never been 
observed. 

The examination in a case of hemianopia may (roughly) be 
conducted in the following manner : The patient is to be placed 
at a distance of about two feet from the examiner, and, if the 
right eye is to be examined, asked to cover his left eye with his 
hand, while with the right eye he fixes the left of the examiner 
who covers his own right eye. The examiner then holds up 
his finger between the patient and himself, and moves it in dif- 
ferent directions as far as the border of his own field of vision, 
the patient at the same time being asked how far out he is able 
to see the finger. The examiner is thus enabled to notice every 
motion of the patient's eye toward the object, and, judging 
from his answers, can compare the patient's field of vision with 
his own. Instead of the finger, a small piece of white paper 
fastened on a dark penholder may be used in a similar way. 
These tests should be made in a good light (Donders, Gow- 
ers). 



DISEASES OF THE OPTIC NERVE. 



37 



The more extensive defects can always be found out by 
this method ; for slight ones a perimetric examination is in- 
dispensable. An accurate determination of the field of vision 




Fig. 4. — Field of Vision of the Left and Right Eye. (After Forster.) 

with the help of the perimeter can only be attained by prac- 
tice. A description of the instrument and its use is here 




Fig. 5.— Field of Vision of the Left and Right Eye in Left-sided Hemianopia. 

(After Gowers.) 

not necessary. Figs. 4 and 5 illustrate (1) the normal fields 
of the left and right side ; (2) the fields in a case of left-sided 
hemianopia. 



38 DISEASES OF THE CRANIAL NERVES. 

LITERATURE. 

Oppenheim. Die oscillirende Hemiopia bitemporalis als Kriterium der basalen 
Hirnsyphilis. Berliner klin. Wochenschr., 1887, No. 36. 

Freud. Ueber Hemiopie im fruhesten Kindesalter. Wiener med. Wochenschr., 
1888, No. 32. 

Silex. Voriibergehende Hemiopie nach Keuchhusten. Berliner klin. Wochen- 
schr., 1888, No. 42. 

Wilbrand. Die hemiopischen Gesichtsfeldfonnen und das optische Wahrneh- 
mungscentrum. Atlas hemiopischer Defecte, Wiesbaden, 1890. 

Uthoff. Deutsche Med. Ztg., 1890, No. 10. 

Wollenberg. Hemiopie als Folge von Tumoren in der hinteren Schadelgrube. 
Arch. f. Psych., 1890, xxi, No. 3. 

Leyden. Ueber die hemiopische Pupillenstarre Wernicke's. Deutsche med. 
Wochenschr., 1892, No. 1. 

Wilbrand. Ein Fall von Seelenblindheit und Hemiopie mit Sectionsbefund. 
Deutsche Zeitschr. f. Nervenhk., 1892, ii, No. 5 u. 6. 

The so-called flitting scotoma (amaurosis partialis fugax, or 
temporary hemianopia) has in all probability also to be regarded 
as an affection of the center for vision. The disturbance comes 
on in paroxysms. At first a dark spot appears in the field of 
both eyes, which increases in a crescentic or horseshoe form. 
It begins to scintillate and becomes bounded by a bright zig- 
zag line of brilliant colors. If this has after fifteen or twenty 
minutes reached the border of the field of vision, it disappears 
from the center toward the periphery and the field clears up 
again. Most probably in all cases the affection is bilateral. 
The attacks, which last from a half to three quarters of an 
hour, occur with variable frequency, sometimes only once dur- 
ing the whole life, and it is interesting to note that they are 
almost always associated with attacks of migraine. Of the 
causes nothing is known, although the belief that hard mental 
workers are especially prone to it is not without foundation ; 
but there are numerous cases in which we are reduced to re- 
garding sexual and alcoholic excesses, cold, etc., as astiological 
factors. As we are not acquainted with any remedies for the 
disease, we have to be satisfied with prescribing tonics and 
strengthening diet, quinine, and, above all, mental as well as 
bodily rest. The so-called night terrors of children are prob- 
ably to be regarded as due to irritation in the optic center 
(Soltmann). 

The nature and the seat of those forms of amblyopia which 
develop under the influence of hysteria and of certain toxic 
substances are still obscure. 



DISEASES OF THE OPTIC NERVE. ?g 

To this class of substances belong more especially alcohol, 
tobacco, and lead. 

The alcoholic amblyopia is the most frequent form. In the 
mildest cases it manifests itself as a simple central amblyopia 
without distinct scotomata, without disturbances in color vision, 
and without contraction of the visual field ; whereas in the most 
serious forms, which may occur after excessive indulgence in 
spirits, especially in persons of previously moderate habits, 
there may be an acute, almost total blindness. After the recur- 
rence of such attacks a more severe form of atrophic disease of 
the optic nerve may develop, with which is associated discol- 
oration of the whole disk. Central colored scotomata and sim- 
ple scotomata, disturbances in color sense in the whole visual 
field, are then not rare. The ophthalmoscopic examination 
does not reveal anything very characteristic. Vision rarely 
becomes less than -^ to ^, and complete recovery even in the 
most marked cases is possible. The few examinations of the 
optic nerve which have been made after death seem to indicate 
that alcohol exerts a directly injurious action upon the nerve 
itself. The latter has several times been found in a state of 
fatty degeneration with or without compound granular cor- 
puscles and thickening of the interstitial tissue which contains 
the vessels (Erismann, Leber, cf. lit.). Since it has recently 
also been shown that alcohol can act in a similar way upon the 
peripheral nerves this pathological condition is more easily 
understood. 

Similar in its development and in its course is the so-called 
tobacco amblyopia, which, cceteris paribus, is, however, more 
rarely met with than the alcoholic form, and is more benign, 
inasmuch as it usually passes off after the cause is removed. 
The diagnosis is, as a rule, easy enough, as other signs of 
chronic nicotine poisoning (digestive disturbances, palpitation 
of the heart, insomnia) are rarely wanting. The disease seems 
only to occur among those who use tobacco in some form or 
other, in smokers or chewers, while the workers in tobacco, 
who are exposed to the inhalations of the tobacco dust and of 
a certain amount of nicotine, seem, so far as experience goes, 
not liable to the complaint. 

The one form of amblyopia which has been more carefully 
studied than any other, but which nevertheless is not much 
better known or understood than the affections which we have 
just treated of, is lead amblyopia (amblyopia saturnina), in 



40 



DISEASES OF THE CRANIAL NERVES. 



which the field of vision may remain normal or in which there 
may have developed central scotomata or contraction of the 
visual field. Pronounced neuritis, with decided swelling of 
the disk and with peripapillary haemorrhages, has been ob- 
served, and the termination in complete amaurosis is not rare. 

Under certain still unknown conditions a sudden bilateral 
blindness may develop without previous decrease of vision — 
amaurosis saturnina. It is commonly preceded by lead colic. 
The affection, which bears a certain resemblance to the amau- 
rosis of uraemia, may sometimes improve with remarkable 
readiness after the removal of the injurious cause. 

In a given case we should, for the sake of confirming our 
diagnosis, never fail to search for other cerebral symptoms 
common to chronic lead poisoning, such as epileptiform attacks, 
hemiplegia, speech disturbances, and so forth. 

About the relative frequency of the disease no definite 
statement is possible, nor do we know which particular occu- 
pation in the lead industry is the most dangerous, or after how 
long an exposure eye trouble develops in lead workers. The 
role which the so-called individual predisposition plays in this 
connection seems as important as it is obscure. 

In the treatment of the alcoholic amblyopia, local bleeding 
with Heurteloup's cups, active purgation, diaphoretics, and later 
strychnine injections are of service. In tobacco amblyopia the 
treatment is the same, but bleeding may be dispensed with. 
In the saturnine form purgatives are indicated, also opium and 
subcutaneous injections of morphine. In all cases, however, 
the prompt and permanent removal of the injurious agent is a 
sine qua non ; where this can not be done the outlook for re- 
covery is always very doubtful. 

Besides the substances mentioned, quinine, bisulphide of 
carbon (Becker, Centralblatt f. prakt. Augenheilk., 1889, p. 138), 
and mercury may lead to disturbances of sight, which in their 
course resemble those just described. 

LITERATURE. 

Graefe-Saemisch. Handbuch der gesammten Augenheilkunde., Bd. v, Theil v. 
Leber. Die Krankheiten des Sehnerven, pp. 757 et seq. Leipzig, 1877. 
Forster. Beziehungen der Allgemeinleiden u. Organerkrankungen zu Veran- 

derungen u. Krankheiten des Sehorgans. Leipzig, Engelmann, 1877. 
Plobin. Des troubles oculaires dans les maladies de l'encephale. Paris, Bail- 

liere, 1880. 
Magnus. Die Blindheit, ihre Entstehung und Verhutung. Breslau, Kern, 1883. 



DISEASES OF THE OPTIC NERVE. 4I 

Schoeler und Uthoff. Beitrage zur Pathologie des Sehnerven und der Netz- 

haut bei Allgerneinerkrankungen. Berlin, Peters, 1884. 
Jacobson. Beziehungen der Veranderungen u. Krankheiten des Sehorgans zu 

Allgemeinleiden u. Organ-Erkrankungen. Leipzig, Engelmann, 1885. 
Peltesohn. Ursachen u. Verlauf der Sehnervenatrophie. Centralbl. f. prakt. 

Augenheilk., pp. 45, 75, 106. 1886. 
Bergmeister. Die Intoxicationsamblyopien. Wien, 1886. 
Nettleship. Lancet, July 16, 1887 (Quinine Amblyopia). 
Uthoff. Untersuchungen iiber den Einfluss des chronischen Alkoholismus 

auf das menschl. Sehorgan. V. Graefe's Arch., Bd. xxxiii, Abthl. 1. 
Deutschmann. Ueber Neuritis optica, bes. die sogen. Stauungspapille. Jena, 

Fischer, 1887. 
Siemerling. Ein Fall von gummdser Erkrankung der Hirnbasis mit Betheiligung 

des Chiasma. Arch. f. Psych., xix, 2, pp. 401 et seq. 1888. 



CHAPTER III. 



DISEASES OF THE NERVES SUPPLYING THE OCULAR MUSCLES 1. E., ; 

.THE THIRD (MOTOR OCULl), THE FOURTH (PATHETICUS), AND 
THE SIXTH (ABDUCENS). 

The third nerve emerges from the brain at the inner margin of 
the cms close to the anterior border of the pons; it passes obliquely 
forward and outward, reaches the outer wall of the cavernous sinus, 
enters it, and then divides into two branches, which, passing through 

the sphenoidal fissure 
qua 



enter the orbit. The 
upper division, which 
supplies the levator 
palpebrse superioris 
and the rectus supe- 
rior, is the smaller of 
the two. Of the three 
branches of the lower 
division, the one sup- 
plying the inferior ob- 
lique is the longest; 
the two others, one of 
which goes to the in- 
ferior rectus, the oth- 
er to the internal rec- 
tus, are shorter. The 
-Cross Section through the Region of the . , , . . 

ant. Corpora Quadrigemina. longest branch, that 

qu. a. anterior corpora quadrigemina ; g.c. gray matter around inienor OD- 

the aqueduct of Sylvius; aq, aqueduct of Sylvius; nlll lique, gives off a 
nucleus of the third nerve ; hi. posterior longitudinal bun- gftort root to the Clli- 
dle ; r. k. red nucleus (tegmentum) ; sn, substantia nigra , . , ^ . 

(locus niger) ; /, cerebral peduncle. ai T ganglion, the fila- 

ments of which are 
distributed to the ciliary muscle (tensor choroideae) and to the con- 
strictor of the iris (sphincter pupillse) ; consequently these intrinsic mus- 
cles of the eyes also are innervated by the third nerve, while the di- 
lator pupillae, on the other hand, is provided for by the sympathetic. 
42 




DISEASES OE THE MOTOR NERVES OF THE EYE. 



43 



The nuclei of the third nerve, a column of multipolar ganglionic 
cells, lie above the posterior longitudinal bundle, between it and the 
aqueduct of Sylvius, and the root fibres coming from them divide 
into several fasciculi, pierce the posterior longitudinal bundle, the 
tegmentum, with the red nucleus and the substantia nigra, and 
emerge from the brain at the place shown above (cf. Fig. 6). 

Experimental as well as clinical observations seem to indicate 
that in the collection of ganglionic cells of this nerve nucleus there 
exist three centres, the anterior of which is the centre for the ciliary 
muscle (accommodation) ; the next the centre for reflex stimulation 
of the iris by light ; the third, by far the largest, the centre for the 
extrinsic ocular muscles (Gowers). Observers, however, by no 
means agree with regard to the number and position of the indi- 
vidual oculo-motor nuclei or centres. The view held by Gowers is 
diagrammatically illustrated in Fig. 7. 

That there exists a cortical centre for the ocular muscles and the 

levator palpebrarum is beyond question ; nothing certain is, however, 

known about its situa- 

Q 



tion ; most probably it 
lies in the upper or low- 
er parietal lobe (cf. Ex- 
ner, Untersuchungen 
iiber die Localisation 
der Functionen in der 
Grosshirnrinde des 

Menschen. Wien, Brau- 
miiller, 1881, p. 42). 

The fourth, the 
trochlear or pathetic 
nerve, is the smallest of 
the cranial nerves, but 
has the longest course 




fig. 7. — dlagramatic longitudinal section through 
the Pons with the Nuclei of the Ocular nerves. 
(After Gowers. ) C. Q. Corpora quadrigemina ; a a' b b' 
and c c' represent the centres and the nerve-fibres ; a, for 
accommodation, b, for the reflex activity of the iris, c, 
for the extrinsic ocular muscles ; all three are contained in 
the oculomotorius. IV, pathetic. VI, abducens. 



within the skull cavity. It leaves the 
brain close behind the corpora quadrigemina at the upper sur- 
face of the valve of Vieussens ; from here it takes a lateral 
and downward course, winds around the outer side of the crus 
cerebri, and reaches the base of the brain. Its course is now forward ; 
piercing the dura mater behind the anterior clinoid process, it reaches 
a small channel of the cavernous sinus, and runs alongside of the 
third to the sphenoidal fissure, pierces its fibrous membrane, and 
finally enters the superior oblique muscle. 

The nucleus of the fourth lies behind the collection of cells 
from which emanates the third nerve (Wernicke), to the ventral side 
of the aqueduct of Sylvius, on the posterior longitudinal bundle, in 
the gray matter around the aqueduct. From this nucleus the root 



44 



DISEASES OF THE CRANIAL NERVES. 



originates, which, passing to the mesial side of the descending root 
of the fifth (Fig. 8, Vd), extends as a round bundle (IV) to the pos- 
terior corpus quadrigeminum ; in the substance of the valve of Vieus- 
sens it is crossed by the nerve of the opposite side, and emerges 
finally in the above-described manner on the side opposite to that in 
which its nucleus is situated. 

The sixth nerve, the abducens, leaves the brain at the poste- 
rior margin of the pons, between it and the anterior pyramid. It 




Fig. 8.— Cross-section through the Region of the Tegmentum. (After Schwalbe.) 
d, patheticus-crossing. IV, Patheticus at its exit. IV cross-section of the patheticus in 
its course to the nucleus. V.d. descending root of the trigeminus (cross-section) ; aq, 
aqueduct, g . c, central gray substance around the aqueduct, s.f, substantia ferrugin. b, 
sup. peduncle of cerebellum crossing at c \ r, raphe ; f.r. formatio reticularis ; h.l. pos- 
terior longitudinal bundle. 



takes at once a forward course and passes into the cavernous sinus, 
piercing its posterior wall ; it then runs, surrounded by the dural 
sheath, alongside of the internal carotid, and, emerging through the 
sphenoidal fissure, enters the external rectus, in the substance of 
which it breaks up into branches. 

The nucleus of the abducens, which was at one time thought to 
be connected with the root of the facial nerve (hence the facial- 
abducens nucleus of Meynert and Stilling), lies in the floor of the 
fourth ventricle, from which it is separated by the ependyma. The 
abducens root, passing through the peduncular portion of the pons 
to the outer side of the pyramids into the tegmental region of the 
pons to the median side of the upper olive, finally enters this nucleus 
(cf. Fig. 9). The tegmentum behind the lemniscus is divided into 
three parts by the abducens (and facial) root, the inner two of 
which Meynert has called the motor region of the tegmentum. 



DISEASES OF THE MOTOR NERVES OF THE EYE. 



45 



The affections of the nerves supplying the ocular muscles 
belong, strictly speaking, also to the domain of ophthalmology. 
Since, however, they are of such importance for the diagnosis 
and the prognosis in certain nervous diseases (e. g., tabes), it is 
necessary to devote a few pages at least to the description of 
their symptoms and the proper methods of examination. 

The independent diseases of the muscles of the eyes may 
be of a paralytic or of an irritative (spastic) nature, the latter 



nVI 

nVni VII I VILa THa 

III I 



yir 




vni 'A 



Fig. 9. — Cross-section through the Pons. (After Schwalbe.) nVI, abducens nu- 
cleus. VI, abducens. O.S., upper olive. aV, ascending root of trigeminus. nVII, 
nucleus of facial, n VIII, auditory nucleus (so-called external nucleus). VII, emerging 
portion of facial root, po, transverse fibres of the pons which are divided into superficial 
po x and deep po 2 . py, pyramidal tract. 



class, however, being by far the less frequent of the two. Their 
seat may be central or peripheral, although we should state 
that an undoubted central affection of the abducens and of the 
patheticus has never been observed. Of oculo-motor paralysis, 
we are acquainted with a peripheral and a central form. 

A peripheral affection may have its seat in the stem or in 
its branches ; a central, in the nucleus or the (supposed) cortical 
center of the nerve. The former will be characterized by the 
absence of all cerebral symptoms, which, in the central form, 
are almost always present. It can be brought about by patho- 
logical changes in the orbit, in which case the eyeball not 
infrequently protrudes and becomes immobile. Further, it 
may develop as a rheumatic paralysis from exposure to cold 



46 DISEASES OF THE CRANIAL NERVES. 

(a frigore)\ also in constitutional syphilis, in diphtheria and 
other acute infectious diseases, in meat poisoning, and as the 
result of alcoholic excesses ; exceptionally it is seen after trau- 
matism. In one of my cases a man was kicked by a cow in 
his right eye ; after the acute symptoms had passed off, a pa- 
ralysis of the levator palpebras superioris remained for months. 
Power of vision was not interfered with. 

The central paralysis is met with in the course of meningi- 
tis, multiple sclerosis, progressive bulbar paralysis, and, above 
all, locomotor ataxia. It rarely affects all the ocular mus- 
cles at the same time, but either the extrinsic or the intrinsic 
alone (cf. Knies, Ueber die centralen Storungen der willkiir- 
lichen Augenmuskeln, Arch, fiir Augenhk., 1891, xxiii, 1, p. 
19). Although the diplopia of tabetics is neither a constant 
nor a pathognomonic symptom of the disease, the occurrence 
of transient double vision in otherwise apparently healthy per- 
sons ought always to make us suspicious, and ought to induce 
us to subject the patient to a more careful examination. The 
nature as well as the anatomical seat of this oculo-motor pa- 
ralysis occurring in tabes is entirely obscure. A monocular di- 
plopia may occur in hysterical patients ; owing to disorders of 
accommodation two or more images are thrown upon the 
retina (Bouveret et Chapetot, Revue de med., 10 Sept., 1892, 
p. 728 ; and Duret et Dujardin, Sur la diplopie monoculaire 
comme symptome cerebral, Journal des sciences med. de 
Lille, 1892). 

Of the cortical oculo-motor paralysis we know little or 
nothing ; the only well-established fact is that an isolated 
paralysis of the levator palpebras superioris may be associated 
with cerebral affections — for instance, with a cerebral haemor- 
rhage, but the location of the center is not known. Grasset 
and Landouzy thought it to be in the second temporal convo- 
lution (the pit courbe of the French writers), but Charcot and 
Pitres have adduced important reasons against this view. 
Lately the subject has again been taken up by Lemoine 
(Revue de med., 1887, vii, 7)- This " blepharoptosis cerebra- 
lis " needs much further investigation. 

Isolated ptosis may be unilateral or bilateral ; it may be ac- 
quired or congenital. Of the latter form Siemerling has pub- 
lished a case, with autopsy, in which he found degenerative 
changes in the main cell group of the ventral as well as the 
dorsal oculo-motor nucleus (Arch. f. Psych., 1892, xxiii, 3, p. 



DISEASES OF THE MOTOR NERVES OF THE EYE. 47 

764). It is interesting to note that some patients with ptosis 
are able to open their eyes if they put into activity certain 
muscles supplied by the trigeminus — for example, the muscles 
of mastication. 

Acquired ptosis is not always due to an affection of the 
third nerve, but may be the result of a primary atrophy of 
the levator palpebrse superioris. Fuchs has reported a num- 
ber of such cases (Arch. f. Ophthalm., 1890, xxxvi, 1, p. 234). 
Dutil has described two cases of ptosis in the same family. 
(Note sur une forme de ptosis non congenital et hereditaire, 
Progres med., 1892, 2 S., xvi, 46). The duration of the dis- 
order varies. I have notes of several patients in whom ptosis 
existed for years, and in whom no other, spinal or cerebral, 
symptoms developed. A very complete paper on the astiology 
and the serological diagnosis has lately been published by Dali- 
chow from Senator's clinic (Zeitschr. f. klin. med., 1893, xxii, 

4, 5)- 

In studying the symptoms of the paralyses of the ocular 
muscles we shall first consider those of the oculo-motor pa- 
ralysis, more especially of the complete form, in which all 
branches of this nerve are implicated. 

The upper eyelid droops completely, and the eye can only 
be opened slightly by the aid of the frontalis ; the movements 
of the eyeball are also at fault ; the eye, deviated outward as 
it is, can not be moved toward the nose ; similarly any up- 
ward motion is impossible, as such depends upon the supe- 
rior rectus and the inferior oblique. On the other hand, the 
outward movements are unhampered (rectus externus), while 
the downward motion is performed by the superior oblique, 
the pure action of which can here be well studied, the rectus 
inferior, which otherwise also assists in the downward motion 
of the bulb, being now inactive. 

From the different directions of the axes of the two eyes 
there results a very apparent symptom, namely, strabismus, 
which may be convergent or divergent, according to the mus- 
cles affected. This strabismus, due to paralysis of the ocular 
muscles (paralytic), differs from that caused by spasm (spas- 
modic), inasmuch as (1) in the latter the deviation exists with 
all movements, while in the former only with those which call 
into action the paralyzed muscle ; (2) in spasmodic strabismus 
the secondary deviation of the sound eye, of which we shall 
presently speak (cf. p. 51), does not occur. 



48 DISEASES OF THE CRANIAL NERVES. 

The double vision, " diplopia," which is associated with 
strabismus, is especially marked at the beginning of the dis- 
turbance, before the patient has learned to suppress the " false 
image " seen with the affected eye, and only to pay attention 
to the " true image " seen with the healthy one (cf. Amon, 
Ueber Diplopie, Munchener med. Wochenschrift, 1890, 46). 
At first these double images cause him much annoyance, until 
later on he learns to close the affected eye by contraction of 
the orbicularis, or to put the head into a position in which the 
affected muscle is not called into play. By these devices he 
not only avoids the unpleasantness of the double images, but 
also the consequences which the erroneous projection of the 
visual field entails, namely, a peculiarly disagreeable feeling of 
dizziness, the so-called ocular or visual vertigo, to which we 
shall have occasion to refer again. 

With reference to the pupillary symptoms we must keep in 
mind the reactions present in a normal eye: the pupil reacts 
directly to changes between light and darkness, contracting if 
light is thrown into the eye, and indirectly in that the pupil of 
one eye dilates if the other is covered ; it also reacts on mo- 
tions of convergence and on forced accommodation, contract- 
ing in either case. All these reactions are lost in complete 
paralysis of the third nerve. The pupil is moderately dilated 
and gives no response to the influence of light or accommoda- 
tion ; if the paralysis is incomplete, and either the sphincter of 
the iris or the ciliary muscle, or both, are intact, so that in the 
latter case only the extrinsic muscles do not perform their 
function, the size of the pupil can vary and accommodation be 
retained. 

The reflex immobility of the pupil (Erb), also called the 
" Argyll-Robertson pupil " — that is, where the pupil has lost 
its reaction to light impressions (reflex), but has retained its 
power of accommodation — is very frequently observed in 
tabetics. Besides this, the pupil in tabes is often very small, 
pin-head pupil — spinal myosis. 

Inequality of the pupil, anisocoria, is also seen in the course 
of tabes, in general paralysis, in hemicrania, optic atrophy, 
separation of the retina, accommodation paralysis, etc. Recke, 
in the ophthalmological clinic of Magnus in Breslau, has lately 
pointed out that this symptom need by no means have the 
ominous significance which has formerly been attributed to it, 
but that not infrequently it is found associated with astigma- 



DISEASES OF THE MOTOR NERVES OF THE EYE. 40 

tism, myopia, and with presbyopia, especially in men, without 
the existence of any central disease (Deutsche med. Wochen- 
schrift, 1893, 13). 

LITERATURE. 

Leeser. Die Pupillarbewegung in physiologischer und pathologischer Bezieh- 

ung. Wiesbaden, Bergmann, 1882. 
Heddaeus. Die Pupillarreaction auf Licht, ihre Priifung, Messung und klinische 

Bedeutung. Wiesbaden, Bergmann, 1886. 
Konigstein. Physiologie und Pathologie der Pupillarreaction. W T iener Klinik, 

1888, Heft 4. 
Heddaeus. Reflexempfindlichkeit, Refiextaubheit und reflectorische Pupillen- 

starre. Berliner klin. Wochenschr., 1888, 17, 18. 
Seggel. Arch. f. Augenhk., 1892, xxvi, 2, p. 151. 

Paralysis of the abducens, unilateral or bilateral, which also 
comparatively frequently accompanies locomotor ataxia, often 
constituting here the only initial symptom for a long time, is to 
be recognized by noticing that the eye, which is slightly turned 
inward, can not be moved outward, while all the other move- 
ments are unimpeded. In exceptional cases this is found asso- 
ciated with facial and trigeminal paralysis. The condition is 
usually congenital. Bernhardt (cf. lit.) has reported cases of 
this kind, and Mobius in an extensive article gives a careful 
study of the infantile nuclear degeneration, and has especially 
called attention to the fact that a large proportion of all ocular 
palsies are congenital or acquired in early life (Miinchener 
med. Abhandl., 1892, 6. Reihe, Heft 4). 

Unilateral paralysis of the abducens has also been ob- 
served after fracture of the base of the skull (Kohler, Ber- 
liner klin. Wochenschr., 1891, 18). 

Unilateral paralysis of the patheticus, which supplies the 
superior oblique muscle, is always difficult to recognize even 
when the muscular system of the other eye remains perfect, 
and can only be diagnosticated after an examination of the na- 
ture of the double images. When there is paralysis of the 
oculo-motorius in the other eve a diagnosis is impossible. The 
examination ought to be made by an ophthalmologist in order 
to establish the absence of power in the superior oblique (cf. 
Halm, Beitrage zur Trochlearislahmung. Tubingen, Moser, 
1888). Extremely rare is the bilateral patheticus paresis, which 
has been noted in some cases of tumor of the pineal gland. 
The anatomical conditions directly underlying it are not 
known (Remak). 



50 



DISEASES OF THE CRANIAL NERVES. 



A paralysis of the patheticus, superadded to a paralysis of 
the oculo-motorius, may be recognized by the absence of the 
characteristic rotation around the sagittal axis, which would 
otherwise occur on looking down (Wernicke). 

If several muscles of one eye which are supplied by differ- 
ent nerves are paralyzed, or if there exist paralysis of the mus- 
cles of both eyes, we speak of an ophthalmoplegia (Hirschberg, 
Mauthner), and we distinguish an external ophthalmoplegia if 
only the extrinsic, and an internal ophthalmoplegia if only the 
intrinsic, muscles of the eye are paralyzed (sphincter, dilator, cil- 
iary muscle). The so-called ophthalmoplegia progressiva (von 
Graefe) will be described in the eleventh chapter of this' part 
under the name of poliencephalitis superior (Wernicke). Quite 
lately attention has been drawn to a so-called recurrent pa- 
ralysis of the third nerve, of which Mauthner has analyzed four- 
teen instances. This disease is characterized by the fact that 
only one, and always the same, oculo-motor becomes affected, 
and that the paralysis is always complete — that is, takes in all 
the branches. Females, especially those of a nervous or hys- 
terical temperament, seem more predisposed to the affection 
than males. The duration of the individual attacks varies 
from one, three, four, to even six months. They may recur 
after an interval of from four weeks to a year. Other nervous 
symptoms — migraine, vertigo — may or may not accompany 
them. The attacks may recur during the whole life of the 
patient, and even in the intervals traces of paralysis may re- 
main (Mobius, Remak). Whether there are instances in which 
the disturbance is only functional, or whether in all cases there 
exists a distinct organic basis, we are with our present mate- 
rial unable to decide definitely, and we are equally in the dark 
with reference to the seat of the affection, as to whether it is 
of peripheral or of central origin. That there are instances 
where the former is true is proved by a case published by 
Richter (cf. lit.), where a new growth in the nerve itself was 
found. 

In a suspected paralysis of the ocular muscles we endeavor 
to make out in our examination any defects in the mobility of 
the eyeball. For this purpose the patient is asked to follow with 
his eyes the finger of the examiner in different directions without 
moving his head. In this way every asymmetry in the move- 
ments of the two eyes can be noted. If the mobility in the direc- 
tion of the action of the affected muscle is defective ("primary 



DISEASES OF THE MOTOR NERVES OF THE EYE. § l 

deviation "), nystagmus-like twitching is sometimes observed 
on attempts at extreme rotation in that direction. But it 
may happen that the paresis of a muscle is not recognized if its 
innervation is particularly strong ; then we have in the corre- 
sponding muscle of the other eye so abnormal an innervation 
that in the latter the effect is excessive, and we get a so-called 
" secondary deviation " of the sound eye. This can easily be 
demonstrated if the presumably healthy eye is first covered 
with the hand and the patient endeavors to fix with the paretic 
eye a point which it can not reach at all or only with the ut- 
most exertion. If, then, the fixing eye is covered, we observe 
whether the healthy eye be in a proper position or not, and 
shall find that the latter has been moved too far in the desired 
direction. If this method does not give any satisfactory re- 
sults, we have to examine into the nature of the double images. 
One eye of the patient having been covered with a colored 
glass, he is asked to follow with his eyes (of course, again with- 
out moving his head) the flame of a candle which is moved to 
and fro. If there exists paralysis or paresis in one eye, the 
patient complains of seeing, on the side toward which the 
affected muscle moves the eye, two flames, which become the 
farther apart the more the affected muscle is exerted. But if 
now, for instance, the patient looking toward the left complains 
of diplopia, this may be due to paralysis of the left external or 
the right internal rectus, as both of these muscles move the eye- 
ball to the left. To determine which of these two is not perform- 
ing its function properly, we must ascertain from the patient 
whether the double images are homonymous or crossed — that 
is, whether the colored picture be on the same or on the oppo- 
site side to the eye covered with the colored glass (homony- 
mous and crossed diplopia respectively). In the former case 
the abducens (rect. ext.) is the nerve affected ; in the latter the 
oculo-motorius (rect. intern.). For a minute study of the double 
images the reader is referred to the plates and the work of 
Landolt, of Paris, which has been translated into German by 
Magnus (Landolt-Magnus, Breslau, Kern, 1887 ; also Landolt, 
Les champs de fixation monoculaires, le champ de fixation 
binoculaire, etc., Arch. d'Ophthalm., 1893, No. 5). 

The associated lateral movements of the eye to the right 
and to the left may be interfered with in the following ways: 

1. There may exist a so-called conjugate deviation of the 
eyes — that is, a permanent fixation of both eyeballs to one side — 



52 



DISEASES OF THE CRANIAL NERVES. 



which can only be overcome, and then but temporarily, by the 
strongest effort. We shall refer to this symptom again in our 
account of hemiplegia. 

2. Motion of both eyes toward one side may be permanent- 
ly lost. In this case we have a paralysis of the abducens of the 
one and paralysis of the internal rectus of the other side, and 
the eyes are turned not toward the affected but toward the op- 
posite side. In such cases the lesion is situated in the lateral 
portion of the pons, near the abductor nucleus. If the centres 
of both sides which lie close together are paralyzed, the eyes 
which are fixed in the middle can be moved neither to the 
right nor to the left, but only upward and downward, the up- 
per eyelid moving normally (Wernicke). 

3. The upward and downward motion of the eyes may be 
lost and only the lateral motion be possible. This form of the 
associated ocular palsy, in which also both upper lids may be 
paralyzed, is caused by a lesion of the centres situated in the 
central gray matter of the third ventricle and the aqueduct of 
Sylvius — that is, in the region of the oculo-motor nucleus. If 
this be accompanied by a hemiplegia, we are justified in diag- 
nosticating a lesion of the pyramidal tract at the level of the 
upper corpus quadrigeminum, the posterior commissure, and 
the adjoining portion of the optic thalamus (Wernicke). 

The treatment of the ocular paralyses is very problemat- 
ical, and rarely produces unquestionable results. Usually a 
trial is made with iodide of potassium, a course which may 
be justified if there is a history of syphilis ; but this drug is 
frequently of no avail whatever. Electricity is used either 
by applying one electrode over the closed lid of the diseased 
eye and the other over the base of the neck, so as to pass the 
current through the whole course of the eye muscles, or by 
allowing the current to pass transversely through the head 
from one mastoid process to the other. Medium-sized elec- 
trodes should be used and a weak current be applied about 
four times a week, each session occupying from one to two 
minutes. Now and again after prolonged galvanization we are 
really fortunate enough to perceive an improvement in the 
paralysis, or even to see it disappear. That much of this is to 
be attributed to the treatment seems doubtful, if we remember 
that it is utterly impossible to stimulate the ocular muscles 
with the current ; for the same reason an electrical examina- 
tion in ocular palsies is impossible (cf. Hirt, Lehrbuch der 



DISEASES OF THE MOTOR NERVES OF THE EYE. 53 

Electrodiagnostik und Elcctrotherapie, Stuttgart, Enke, 1893, 

P- 750- 

Passing over the different spasms of the eye muscles which 
occur in some brain diseases, we shall pay attention here only 
to one form with which the neurologist ought to make himself 
familiar, viz., nystagmus. This consists in a to-and-fro motion 
of the eyeballs in a certain plane, usually horizontal (nystagmus 
oscillator ius), which continues on voluntary movements of the 
eyes, but which is itself not under the control of the will. 
These movements are usually present in both eyes, and vary 
quite markedly in frequency and extent, according as the pa- 
tient is made to fix a point or to change the direction in which 
he is looking. The condition is supposed to be due to weak- 
ness of sight of both eyes, dating from early childhood — that 
is, to impairment in the functions of the retina at a time when 
these have an important regulating influence in the establish- 
ment of the normal fixation of the eyes (von Graefe). How- 
ever, there are undoubtedly cases which do not belong to this 
class, for it is a well-known fact that nystagmus may be an oc- 
cupation disease, as it is often observed in miners who have 
to use their eyes in the dark (Schroeter, Mooren, Nieden, 
Foerster, Snell [British Med. Jour., July 11, 1891] ; Priestley 
Smith [ibid., Oct. 15, 1891], and others); and, secondly, it ap- 
pears in the course of certain nervous diseases — perhaps in 
connection with repeatedly occurring cerebral anaemia (Knoll, 
Ueber die nach Verschluss der Hirnarterien auftretenden 
Augenbewegungen — Sitzungsber. d Akademie d. Wissen- 
schaften in Wien, Abtheilung III, 1S86). In both these classes 
of cases sight is often not diminished at all, and some other 
than the one given above must be the underlying cause ; and, 
as a matter of fact, this nystagmus of the miners is simply due 
to overstrain of the eyes in an insufficient light, while the 
nystagmus occurring in the course of nervous diseases, more 
especially of multiple sclerosis, but also of tabes and epilepsy, 
is to be regarded as a symptom and attributed to the same in- 
fluences as the main disease. That nystagmus, finally, may 
also be a symptom of hysteria, and may persist during the 
whole course of the disease, is shown by a case published by 
myself (cf. Deutsche med. Wochenschr., No. 30, 1887, lit.). 
C. S. Freund has observed nystagmus in a case of Basedow's 
disease (Deutsche med. Wochenschr., 1891, No. 3). 



54 DISEASES OF THE CRANIAL NERVES. 

LITERATURE. 

I. Isolated Oculo-motor paralysis. 

Richter. Typisch-recidivirencle Oculomotoriuslahmung mit Sectionsbefund. 
Arch. f. Psych, u. Nervenhk., 1887, xviii, 1. 

Suckling. Brain, 1887, xxxviii, p. 241 (attacks of migraine followed by transi- 
tory oculo-motor palsy). 

Senator. Ueber periodische Oculomotoriuslahmung. Zeitschr. f. klin. Med., 

1887, xiii, No. 3 u. 4. 

Joachim. Fall von periodischer Oculomotoriuslahmung. Jahrb. f. Kinderhk., 

1888, xxviii, 1. 

Bernhardt. Recidivirende Oculomotoriuslahmung. Berliner klin. Wochenschr., 

1889, No. 47. 

Manz. Die recidivirende Oculomotoriuslahmung. Berliner klin. Wochenschr., 

1889, xxvi, No. 34. 
Vissering. Ueber einen Fall von recidivirender Oculomotoriuslahmung. Miin- 

chtner med. Wochenschr., 1889, xxxvi, No. 41. 
Manz. Doppelseitige Oculomotoriuslahmung bei cerebraler Kinderlahmung. 

Wiener klin. Wochenschr., 1892, v, No. 42. 
Goldschmid. Ein Fall von traumatischer totaler Oculomotoriuslahmung. 

Wiener med. Wochenschr., 1893, xliii, No. 7. 
Dalichow. Aetiologie. und atiologische Dingnostik der Oculomotoriusparalyse 

(from Senator's clinic). Zeitschr. f. klin. Med., 1893, xxii. 

2. Palsies of the Eye Muscles in General. 

Mauthner. Die nicht nucleare Augenmuskellahmung. Wiesbaden, Berg- 
mann, 1886. 

Mauthner. Die nucleare Augenmuskellahmung. Wiesbaden, Bergmanm 1886. 

Mobius. Ueber die Localisation der Ophthalmoplegia exterior. Centralbl. f. 
Nervenhk., 1886, ix, No. 17. 

Westphal. Arch. f. Psych, u. Nervenhk., 1887, xviii, 3, p. 846. 

Landolt-Magnus. Uebersichtliche Zusammenstellung der Augenbewegungen 
im physiologischen und pathologischen Zustande. Breslau, Kern, 1887. 

Remak, E. Doppelseitige Trochlearisparese. Neurol. Centralbl., 1888. 

Mauthner. Differentialdiagnostik der Lahmung der Erhebungsmuskeln des 
Auges. Wiener med. Wochenschr., 1888, No. 24. 

Landolt. Une forme particuliere de Paralyse des muscles oculaires. Clermont 
(Oise), 1889. 

Bottiger. Arch. f. Psych., 1889, xxi, 2, p. 517. 

Bernhardt. Zur Lehre von der nuclearen Augenmuskellahmung und ihrer 
Complicationen. Berliner klin. Wochenschr., 1890, No. 43. 

Thomsen (Bonn). Zur pathologischen Anatomie der progressiven Ophthalmo- 
plegic Festschrift. Hamburg, 1891. 

Barth. Beitrag zur chronischen progressiven Ophthalmoplegic Jahrb. d. Ham- 
burger Staats Krankeninstitutes, 1892, ii, p. 100. 

Schlesinger. Augenmuskellahmung nach Herpes zoster. Wiener med. Presse, 
1892, xxxiii, No. 43. 

Stower. Ein Fall von doppelseitiger Augenmuskellahmung. Munchener med. 
Wochenschr., 1892, xxxiv, No. 48. 



DISEASES OF THE MOTOR NERVES OF THE EYE. 55 

S^hlesinger. Zur Diagnose der chronischen nuclearen Ophthalmoplegic Inaug.- 

Dissert., Tubingen, 1893. 
Cheneys, Frederic. Boston Med. and Surg. Journ., June 24, 1893, cxxviii. 
Bach. Centralbl. f. Xervenhk. u. Psych., N. F., 1893, iii, p. 57. 
Roth. Doppelbilder bei Augenmuskellahmung. Berlin, Hirschwald, 1893. 
Barabaschew. Wiener klin. Wochenschr., 1893, vi, Xo. 17. 
Braunstein. Petersburger med. Wochenschr., 1893. 
Dalichow. Die Aetiologie und die atiologische Diagnostik der Oculomotorius- 

paralyse mit Beriicksichtigung der pathologischen Anatomie. Inaug.-Dis- 

sert., Tubingen, 1893. 
Hotz. Arch. f. Augenhk., 1893, xxvi, 3, 4. 
Jackson, Hughlings. Lancet, July 3, 1893, ii. 

3. Paralysis of the Abdncens. 

Purtscher. Traumatische Abducenslahmung. Arch. f. Augenhk., 1888, xviii, 4. 
Bennett and Savill. Brain, July, 1889, xlv u. xlvi (nuclear paralysis of the 

abducens). 
Blocq et Guinon. Sur un cas de paralysie conjugee de la sixieme paire. Arch. 

de med. experim. et d'anat. path., 1891, i. 
Bloch. Statistisch-casuistischer Beitrag zur Lthre von den Abducenslahmungen. 

Inaug.-Dissert., Berlin, 1891 (438 cases of paralysis of the abducens). 



CHAPTER IV. 

THE DISEASES OF THE TRIGEMINAL NERVE. 

The trigeminus, the stoutest of all the cranial nerves, leaves the 
brain by two separate roots — an anterior small, exclusively motor, 
and a posterior larger, the sensory portion. Its point of exit is 
situated at the base of the pons, where the transverse fibres of the 
latter are prolonged into the middle peduncle of the cerebellum. 
Both roots lie in close apposition, and pass into a recess — the cavum 
Meckelii — formed by the dura mater, and situated over the inner 
end of the superior surface of the petrous portion of the temporal 
bone. Here the posterior root forms a somewhat crescentic swell- 
ing — the Gasserian ganglion — from which pass forward the three 
somewhat flattened divisions, the ophthalmic and the superior and 
inferior maxillary nerves,, the last being joined by the smaller motor 
root. These three branches leave the interior of the skull by the 
sphenoidal fissure, foramen rotundum, and foramen ovale, respect- 
ively. 

The trigeminal nerve possesses two nuclei — a motor and a sen- 
sory one. The first — the smaller — is situated in the outer part of 
the tegmentum, and its ganglionic cells are characterized by their 
relatively large size (60 to 70 ^ in the greatest diameter). The 
larger — sensory — nucleus lies external to the motor; in its collection 
of gray matter there are found very small ganglionic cells (20 to 30 /x 
in diameter). 

With regard to the origin of the two roots there exist very 
different views, and but little is definitely known about the sub- 
ject. It can not be doubted that the motor root springs from what 
has been decided upon as the motor nucleus, nor that there exist a 
number of small bundles of fibres which arise high up in the region 
of the anterior quadrigeminal body, and descend outside the aque- 
duct to the level of the exit of the fifth nerve, where they help to 
form the motor root. This is the so-called descending anterior, or, 
as Henle terms it, superior root, the section of which, a crescentic, 
externally convex, internally concave figure, at once strikes the eye 
in frontal sections of the pons (cf. Fig. 8, Vd). That the sensory 
56 



THE DISEASES OF THE TRIGEMINAL NERVE. 



57 



root arises from the above-mentioned sensory nucleus is probable, but 
not certain. On the other hand, it must be remembered that as low 
down as the neighborhood of the second cervical nerve there can be 
demonstrated in the caput cornu posterioris a layer of longitudinal 
medullated fibres, the highly characteristic transverse section of 
which, crescentic in shape, may be followed upward, as it gradually 
increases in size, as far as the level of the exit of the trigeminus. 
Suitable longitudinal sections plainly show that this longitudinal 
bundle forms a large part of the sensory root of the nerve. This is 
the so-called large ascending root of the fifth, the position of which 



n. Vs. 




Fig. 10. — Nuclei of the Trigeminal Nerve. (After Schwalbe.) n. V.s., nucleus of 
the sensory, n. V.m., nucleus of the motor root, d, fibres passing to the raphe. V.s., 
sensory root. 

in transverse section is represented in Fig. n. The cortical area of 
the trigeminus is not definitely known as yet; still, from experiments 
on animals, as also from the few clinical observations which we pos- 
sess, there is reason to conclude that, at least so far as the motor 
portion of the nerve is concerned, it is located in the region of the 
anterior portion of the fissure of Sylvius; as regards the sensory por- 
tion we know nothing". 



We shall divide the affections of the trigeminus into cen- 
tral and peripheral. In the first class we recognize cortical 
and bulbar diseases ; in the second class Ave have to deal with 
either intra- or extra-cranial lesions. The trigeminus being a 
mixed nerve, containing in by far its larger portion only sen- 



58 



DISEASES OF THE CRANIAL NERVES. 



sory, but in its third branch important motor fibers, we are 
obliged, as there may exist in any case conditions of irritation 

or of paralysis, to distinguish 
clinically between hyperes- 
thesia (neuralgia, neuritis) and 
anesthesia of the sensory part 
of the nerve, and between hy- 
perkinesis (spasm) and akine- 
sis (paralysis, paresis) of the 
w^ T motor portion. 




A* * n!a* 

Fig. ii.— Cross-section through the Me- 
dulla Oblongata. (After Schwalbe.) 
a. V, ascending root of the fifth. n.XII, 
nucleus of the hypoglossus. n.X and n.X, 
nucleus of the vagus. XII, hypoglossal 
nerve, fs, solitary funiculus (respiratory 
fasciculus), p, pyramidal tract, o, olive. 
i. N, pyramidal nucleus, f. /. a. , anterior lon- 
gitudinal fissure, n.am, nucleus ambiguus. 



I. The Central Affections 
of the Trigeminus. 

In dealing with the corti- 
cal affections of the trigeminus 
we discriminate between irri- 
tative and destructive lesions 
of the cortical centre. In the 
former case we get spasm, in 
the latter paralysis of the mus- 
cles of mastication. 

Spasm of the muscles of 
mastication (trismus, mastica- 
tory facial spasm — Romberg) 
occurs frequently as a part 
of general convulsions (Sena- 
tor, Petrina, Seligmuller), and much more rarely indepen- 
dently, unaccompanied by other spasms (Lepine, von Pfungen, 
Langer). There are two forms : a tonic, in which the teeth 
are pressed firmly together and the muscles of mastication, 
usually of both sides, are hard as wood to the touch ; and a 
clonic, in which the lower jaw is moved to and fro horizon- 
tally or vertically, and spasmodic masticatory movements are 
induced. In a case in my practice, in an old gentleman who 
had suffered from repeated slight apoplectic attacks, the pa- 
tient for several hours every day goes through well-marked 
chewing movements without eating anything, which at times 
are so vigorous that he often while smoking bites through 
his cigar unintentionally. The origin of the disease is 
often of a reflex nature. Toothache, periostitis of the infe- 
rior maxilla, or face-ache may give rise to it. Sometimes, it 
may be, a cortical affection lies at the bottom of it, but for 



THE DISEASES OF THE TRIGEMINAL NERVE. 



59 



this there is at present no evidence furnished by post-mortem 
examinations. 

Paralysis of the muscles of mastication is, on the whole, less 
frequently observed than spasm. Barlow, Oulmont, and Kirch- 
hoff report cases of it, recording in some only cortical lesions, 
but in others changes in deeper-lying portions of the brain as 
well. All the cases had this one anatomical feature in common, 
viz., that the cortical lesions always occurred bilaterally, thus in 
every case involving both centres. The first instance in which 
a unilateral lesion of the cortex was found was published by 
myself (cf. lit.). It confirms the supposition that the cortical 
motor area of the trigeminus includes the lower third of the 
anterior central convolution and the adjoining portion of the 
second and third frontal convolutions, and demonstrates that 
a unilateral lesion of the cortex (in this case it was left-sided) is 
sufficient to paralyze the muscles of mastication on both sides. 
The lesion was due to the presence of a psammoma the size of 
a filbert, which was situated upon the dura and cortex at the 
spot indicated, causing a depression and softening of the latter. 
The paresis of the muscles of mastication had reached a high 
degree, and was the more interesting from the fact that it was 
accompanied by periodical attacks of pain in the face and 
spasm in the area of distribution of the left facial nerve. Pa- 
resis and paralysis of the muscles of mastication are occasion- 
ally observed among the symptoms due to progressive bulbar 
paralysis and to pseudo-bulbar paralysis. The idea that these 
may develop as the result of a peripheral affection in an isolated 
disease of the motor portion of the third part of the trigeminus 
can not a priori be considered as impossible, but there have 
been up to the present no such cases observed. The differen- 
tial diagnosis between a central and peripheral affection could 
be made only by means of an electrical examination. The 
lesion is central if there are neither quantitative nor qualitative 
changes in the reaction to the faradic and the galvanic cur- 
rents. If such changes, however, exist — for instance, if there 
be the " reaction of degeneration " — the lesion is peripheral. 

Only the latter form of the disease is amenable to treatment 
(by electricity), and then with but slight chance of success. 
Against the central variety we are absolutely powerless. With 
regard to the affection of the nuclei and roots of the fifth nerve 
in the pons, the anatomical relations of which are, as we have 
seen, not as yet sufficiently well understood, we know little 



60 DISEASES OF THE CRANIAL NERVES. 

or nothing. Whether they ever occur independently, or, as 
is more likely, only as concomitants of diseases of other bul- 
bar nerve centres, has not been determined. However, the 
supposition seems justified that the centres in question, in the 
course of certain general diseases of the nervous system — for in- 
stance, in multiple sclerosis and particularly in tabes — are af- 
fected relatively early. Thus Erben reports (Wiener med. 
Blatter, Nos. 43, 44, 1886) that he has observed very trouble- 
some paresthesias of the sense of taste in tabetics occurring in 
paroxysms, beginning in the pharynx. These were especially 
pronounced at the anterior edge of the tongue, and were ac- 
companied by anaesthesia in the second branch of the fifth. 
This condition is presumably to be considered a disease of the 
nerve of taste, being analogous to the so-called gastric crises 
which are attributed to an affection of the vagus centre. A cen- 
tral anaesthesia of the trigeminus may also occur. In its symp- 
toms it would not differ from the peripheral except that it may 
be bilateral. The central nature of the trouble one would infer 
from the simultaneous participation of other nerves, both sensory 
and motor (Romberg). The interference with conduction may 
take place at the base of the brain. 

LITERATURE. 

Romberg. Lehrbuch der Nervenkrankheiten des Menschen. 3. veranderte 

Auflage. Berlin, 1853, p. 367 et seq. 
Senator. Berl. klin. Wochenschr., 4, 1879. 
Petrina. Prager Vierteljahrschrift. Bd. 133. 
Seligmiiller. Archiv fur Psychiatric Bd. vi, p. 825. 
Gerhardt. Festschrift der Wurzburger Universitat. Leipzig, 1882. 
Barlow. Brit. Med. Journ., July 28, 1877. 
Oulmont. Revue mensuelle, 1877. 
Kirchhoff. Arch. f. Psychiatrie. Bd. xi, p. 133. 
Hirt. Zur Localisation des corticalen Kaumuskelcentrums beim Menschen. 

Berl. klin. Wochenschr., No. 27, 1887. 

II. Peripheral Affections of the Trigeminus. 
In its peripheral part the nerve may become diseased inside 
as well as outside of the cranium. If the lesion is one of the 
stem, and extends to all three branches, it may be difficult, in- 
deed impossible, to determine its exact site, because we do not 
as yet possess any means which enable us to definitely decide 
whether the nerve is diseased centrally from the Gasserian 
ganglion, whether the ganglion itself, or, finally, whether the 



THE DISEASES OF THE TRIGEMINAL NERVE. 6 1 

three individual branches are all affected at their exit from the 
skull. 

An affection of the nerve stem to the central side of the 
Gasserian ganglion can only be assumed with any degree of 
certainty if the nerve is diseased in its whole sensory distri- 
bution and if extensive trophic disturbances are also present. 
The affections of the Gasserian ganglion itself (inflammation, 
neoplasm, softening) have as yet but little practical importance. 
If the disease does not take in the stem, but only the terminal 
portions of the individual branches, it is easy to determine the 
seat, and while we have in the intracranial diseases to consider 
those of the finer branches of the nerve in the dura, in the ex- 
tracranial we have the branches distributed to the face and 
those to the nasal cavity. 

A. Intracranial Diseases. 

Headache — Cephalalgia. 

Since it is very likely that there are only exceptional cases 
of idiopathic headache in which the fifth or its terminal 
branches in the dura (cf. page 3) are not implicated, it seems 
advisable to speak of headache here. At the same time we 
must expressly state that we are forced thus to take up differ- 
ent diseases together, which are aetiologically as well as patho- 
logically to be strictly separated from one another. 

The main point to decide in a given case will always be 
whether the headache is to be regarded as merely a symptom 
of another disease or as an affection by itself. Nobody ever 
would assume a headache which occurs at the onset of a severe 
illness — for instance, an acute infectious disease, or in associa- 
tion with organic brain disease (e. g., tumor), or during grave 
disorders of nutrition, anaemia, and chlorosis — to be an affec- 
tion, by itself and treat it as such. These headaches will 
always be considered as a mere symptom of the underlying 
disease ; but when we find an otherwise healthy person suffer- 
ing from protracted or paroxvsmal headache, while on repeated 
careful examination we are unable to discover any other dis- 
ease, then we are forced to assume an independent affection 
and we have to endeavor to determine the following points : 
(a) The seat of the headache ; (b) its peculiarities and its course ; 
(c) its aetiology ; (a 7 ) its appropriate treatment. 

(a) The anatomical situation of the headache can hardly 



62 DISEASES OF THE CRANIAL NERVES. 

ever be determined ; but we are justified, since we do not 
know what part the brain substance takes in it, in believing 
that the sensory terminal branches of the trigeminus in the 
dura (the dura receives at least two branches from the tri- 
geminus) are always implicated, and are thus in some measure 
the seat of the headache. Under what conditions these nerve 
endings are thrown into a state of irritation — a state upon which 
the headache depends — is not well understood, and all we know 
about this question is more or less hypothetical. The most prob- 
able explanation is that the amount of blood in the brain or its 
membranes at the time being is an importantfactor in the pro- 
duction of the morbid condition, whether there be a permanent 
increase or decrease or frequent, perhaps very slight, changes in 
the amount. An increase constitutes what is called cerebral hy- 
peraemia, a decrease cerebral anaemia ; and we assume the former 
condition if full-blooded individuals, who are liable to rushes of 
blood to the head, complain of paroxysmal headache ; the latter, 
if it occur in pale, anaemic patients who are subject to fainting 
spells. However, we do not know anything positive, and we 
shall have occasion to deal more in detail with this in another 
place. Of the greatest interest, and perhaps of the most com- 
mon occurrence, are the fluctuations in the intracranial blood 
pressure, which possibly are the cause of the irritation of the ter- 
minal branches of the trigeminus in the dura and pia. If such 
fluctuations appear frequently, so as to give rise to an unequal 
distribution of the blood in the two halves of the brain, the 
irritability of the sensory endings may become abnormally in- 
creased, so that slight causes are sufficient for the production 
of the pathological condition. The clinical observations even 
go to show that without any demonstrable cause from time to 
time there may develop an increased irritability of these ter- 
minal branches of the fifth, associated with simultaneous fluctua- 
tions in the blood pressure. If the attack of headache thus pro- 
duced is accompanied by vaso-motor symptoms, either of a para- 
lytic or of an irritative nature, it is designated as migraine or 
hemicrania, the latter name being given to those not very com- 
mon cases in which the pain is strictly confined to one side of 
the head. Owing to the vaso-motor disturbances just men- 
tioned, some have been inclined to locate the seat of the disease 
in the sympathetic system, without being able, however, to 
show that the symptoms referable to the sympathetic are not 
perhaps only a secondary result of the pain, and therefore reflex 



THE DISEASES OF THE TRIGEMINAL NERVE. 



63 



in nature (Mobius) ; and until this is actually demonstrated not 
to be the case we are justified in looking upon migraine as be- 
longing to the affections of the trigeminus. In some, as it 
seems, quite exceptional cases, the seat of the headache is to 
be referred to certain muscles, which present at their origin 
and insertion as well as in their course points of tenderness. 
Among these, besides the frontal occipital and temporal mus- 
cles, are the sterno-cleido-mastoid and the upper part of the 
trapezius. This myalgia, which is occasionally produced by an 
unnatural position during sleep, and which is easily diagnos- 
ticated on careful examination, is said under certain circum- 
stances to be the cause of headache. 

{b) With regard to the peculiarities and the course of the 
headache connected with the affections of the trigeminus, we 
know that in its character as well as in its situation it presents 
no inconsiderable number of variations: thus, while one patient 
complains of a dull, boring ache, another describes his pain as 
sharp and burning; while in the one it is worse in the forehead, 
another refers it chiefly to the occiput, vertex, or temples, etc. 
In some instances the patients designate sharply circumscribed 
places of the hairy scalp as the seat of their pain. The head- 
ache also varies much in degree — from a dull sensation of pres- 
sure to a pain which allows of no sleep. In some cases the 
suffering is increased by a touch or a tap on the head, while in 
others it is soothed by a firm bandage around the temples. 
Seldom do we find a headache lasting for days, weeks, or even 
months without interruption ; usually there are times when 
it is less severe or when it ceases completely. There is no 
regularity or uniformity in the occurrence or duration of the 
attacks. Two cases are scarcely ever alike, and almost always 
each presents certain peculiarities of its own : thus in the one, 
slight febrile movements, absent in another, may occur ; one 
patient enjoys a splendid appetite during the most violent pain, 
while another is unable to eat a thing, etc. 

(c) ^Etiological!}', heredity plays a certain role, though this is 
far less important than in the case of migraine. Frequently 
the parents of the patient, especially the mother, have from 
their youth up suffered from headache without attaching much 
importance to it or consulting a physician for it. Mental over- 
work in young people is sometimes a factor, and rapidly grow- 
ing youths not infrequently suffer from headache (cephalasa 
adolescentium). In anaemic and chlorotic conditions, in chronic 



64 DISEASES OF THE CRANIAL NERVES. 

dyspepsia, after acute alcohol intoxication, headache is of com- 
mon occurrence ; it may also be caused by diseases of the 
pharynx and the middle ear (Legal). The aetiological impor- 
tance attributable to errors in accommodation or refraction has 
been pointed out by Bickerton. Certain poisons, if introduced 
into the body for a long period of time, lead to habitual head- 
ache — e. g., lead, tobacco, and others ; the headache found in 
lues and malaria in all probability also belongs under this cate- 
gory. The reflex origin of headache due to affections of the 
nose and the sexual organs, especially the uterus, has only of 
late years been sufficiently appreciated. It is most important 
that the nose should be carefully examined for swellings (Bres- 
gen, Miinchener med. Wochenschr., 1893, No. 5). 

In exceptional cases migraine-like attacks are met with in 
cases of gout, and it would appear as if they were also in some 
way connected with the excretion of uric acid, since it has been 
found that before the attack no uric acid can be detected in 
the urine, while after it the amount is very perceptibly in- 
creased, and later on for a time markedly diminished. The 
polyuria, which occurs frequently after the attack and lasts for 
several hours, with an acid urine, light yellow, almost as clear 
as water, of a very low specific gravity (1.005 to l -°°7)i nas 
been mentioned before. To the fact that migraine-like attacks 
may also occur in the initial stage of tabes and may be of 
importance for the diagnosis and prognosis, we shall have to 
refer later. 

It is difficult, indeed at times impossible, to give a reliable 
prognosis in the cases now under consideration. So far as life 
is concerned, it is always favorable, if the case is of a purely 
functional character— where the headache exists by itself as an 
independent affection, and where it is not to be regarded as a 
symptom of organic disease. The patient recovers from his 
severest attacks comparatively readily, and even after frequent 
repetitions of them it is exceptional that the digestive disturb- 
ances and the loss of strength which these entail induce a really 
serious condition. 

But is the prognosis for recovery as good as for life? To 
this question we must answer without reservation, No. One 
can not deny that the outlook for a complete recovery is, on 
the whole, very bad, and that the chances, cceteris paribus, are 
so much the worse the longer the affection has lasted, and the 
more difficult it is to find any tangible cause for its occurrence. 



THE DISEASES OF THE TRIGEMINAL NERVE. 



65 



The worst cases are those in which the trouble is inherited ; in 
these recovery is very exceptional. At any rate, the prognosis 
in all cases should be guarded, and little should be promised. 
There is hardly any other condition which is so liable to injure 
the physician's authority and the patient's faith in him and his 
medical skill as migraine and habitual headache. On the other 
hand, spontaneous recoveries are not unheard of — a fact which 
we ought to remember, if all our drugs leave us in the lurch. 

(d) The treatment of habitual headache is generally very 
tedious, and puts to a severe test the perseverance not only of 
the patient but also of the physician. It is therefore abso- 
lutely necessary, before undertaking to take charge of a patient 
of this kind, to lay down, after a most careful and minute ex- 
amination, a definite plan of treatment, which must be rigor- 
ously adhered to. It is not sufficient to use to-day one drug 
and to-morrow another, of which we have possibly read in the 
last journal as being effectual against headache, and with which 
we may accidentally obtain a transient good result. The treat- 
ment must rather be systematic, and the outcome of certain 
well-considered conclusions, which we shall now briefly dis- 
cuss. In the first place, we have to decide whether there exists 
some underlying disease which causes the headache. If, as is 
frequently the case, stomach symptoms are present, a stay at 
Carlsbad or Kissingen may do much good. If the acidity of 
the gastric juice is increased, the regular ingestion of alkaline 
drinks or of lukewarm water is indicated. In all cases much 
attention is to be paid to the diet, and the patient should 
especially be warned against overloading his stomach at night. 
The regulation of the bowels is effected by massage or the use 
of large enemata of water, or of small injections of pure gly- 
cerin (5 to 6 cc. — tti lxxx to c. — at a time), or by vegetable ape- 
rients, such as rhubarb. Any degree of constipation may be 
attended with bad consequences. Diseases of the middle ear 
or of the pharynx should be treated by a specialist. If the pa- 
tient have a gouty diathesis, the use of lithium and the regula- 
tion of the diet should constitute the main treatment. The 
eyes should be examined for any possible errors of accommo- 
dation or refraction that may exist, and these, when found, 
should be corrected by means of proper glasses. Cases which 
had resisted all other treatment have been cured in this man- 
ner (Bickerton, Brailey, Weir Mitchell, and others). 

If no coexisting disease can be detected, our chief efforts 

5 



66 DISEASES OF THE CRANIAL NERVES. 

must be directed to building up the general constitution. 
From the cold-water treatment, general faradization (according 
to Beard and Rockwell), franklinization with the Holtz ma- 
chine, systematic gymnastic exercise at home — from any one 
of these measures we may, under certain circumstances, obtain 
the desired result. In some cases lasting advantage has been 
seen from a change of climate, from travel, and a stay in the 
mountains or at the seaside. With regard to the combating or 
the shortening of the attacks, antipyrine, i.o gm. (15 grs.) at a 
dose, or 3 to 4 gm. (45 to 60 grs.) a day, or phenacetin, 0.25 gm. 
(4 grs.) at a dose to 1.25 gm. (20 grs.) a day, may be given. 
The exhibition of these drugs is frequently followed by good 
results, although this is rarely lasting. If vaso-motor changes 
point to the existence of a pathological contraction or dilata- 
tion of the blood-vessels, we may in the former case — in that 
of contraction — resort to the careful administration of nitrite 
of amyl, three to five drops of which are put on a handker- 
chief and given the patient to inhale ; or to the internal use of 
nitroglycerin (one drop of a one-per-cent alcoholic solution 
three times a day). Great care has to be exercised in the ex- 
hibition of the latter drug, and, if the pulse indicate it, we ought 
to begin with minimum doses. Such a precaution is more 
especially necessary if the pulse is full and the arterial wall 
tense, in which case a quarter or half a drop is sufficient as an 
initial dose (Trussewitsch). It is, moreover, not advisable to 
continue its administration any longer than one or two weeks, 
as it is liable to give rise to cerebral symptoms (buzzing in the 
head, vertigo). In the second case — that of vaso-dilatation — 
ergot is indicated, which may either be used in the form of 
hypodermic injections of ergotin (ergotini dialysati, 1.0 — grs. 
15; aquae destill., 4.0 — 3j. Sig. : Half a syringeful) ; or by 
the mouth (extr. secal. corn. (Denzel), 2.0 — ttt, xxx ; aquae cin- 
namomi, 180.0 — 5 v j- Sig. : A tablespoonful every two hours). 
If no such indications are furnished by the condition of the 
blood-vessels, we have to try which medicine will do the most 
good, and may begin with the citrate of caffeine (0.15 — about 
two grains — three times a day), which we have found to be 
effectual. The pasta guarana, 2.0 grm. (grs. xxx) twice a day, 
gives similar results, but often interferes with digestion. Sali- 
cylic acid is in many cases, especially at the onset, followed by 
surprising results, but its continued use is disagreeable to the 
patient on account of its bad after-effects. Application to the 



THE DISEASES OF THE TRIGEMINAL NERVE. 



6 7 



painful spot of an alcoholic solution of menthol (three to twen- 
ty) is often both agreeable and refreshing- to the patient, the 
migraine pencils, also prepared with menthol, having a similar 
effect. This, according to Goldscheider, gives rise to a hyper- 
esthesia to cold which is associated or followed by a diminu- 
tion in the excitability of the sensory nerves. If painful points 
can be discovered on the scalp or on the muscles {vide supra), a 
slight pressure and kneading of the same, later a more energetic 
massage to the head, is advisable. 

Electricity may be used (1) in the form of a constant cur- 
rent passed longitudinally or transversely through the head or 
by applying it to the cervical sympathetic, and (2) in the form 
of the faradic current. In this case it is best for the physician 
to apply his own hand, previously moistened, to the forehead of 
the patient, this taking the place of one electrode. In the other 
hand he holds one of the electrodes, the other being placed on 
the back of the patient's head, the sternum, or some other in- 
different point. With this mode of application, which is called 
the " faradic hand," only very mild currents should be used 
(cf. Hirt, Lehrbuch der Electrodiagnostik und Electrotherapie, 
Stuttgart, Enke, 1893). 

Numerous as are the means at our disposal for combating 
the disease, quite as numerous are the patients who, after hun- 
dreds of unsuccessful trials, give up all medicines and all physi- 
cians. They retreat at the beginning of the attack from the 
world and from their families, darken their rooms, lie down 
quietly, and take simple domestic remedies, among which Rus- 
sian tea with lemon juice has obtained a prominent place. Ab- 
solute rest is what always does most good to all these patients. 
Finally, we should not forget to deprecate, especially here, the 
use of all hypnotics, more particularly morphine, as they never 
do any good, and are often capable of producing serious harm. 

LITERATURE. 

Keller. De la cephalee des adolescents. Arch, de neurol., 16, 17, 1883. 
Rossbach. Nervose Gastroxynsis als eine eigene, genau charakterisirbare Form 

der Dyspepsie. Deutsches Arch. f. klin. Med., xxxv, p. 383, 1884. 
Legal. Ueber eine oftere Ursache der Schlafe-Hinterhauptskopfschmerzen. 

Deutsches Arch. f. klin. Med., xl, 2, 1887 ; Bresl. arztl. Zeitschr., viii, 20, 1887. 
Faust, W. Antifebrin gegen Kopfschmerz. Deutsche med. Wochenschr., No. 

26, 1887. 
Bickerton. On Headache due to Errors of the Refractive Media of the Eye. 

Lancet, August 13, 1887, p. 303. 



68 DISEASES OF THE CRANIAL NERVES. 

Trussewitsch. Ueber Anwendung und Dosirung des Nitroglycerins als Arznei- 

mittel. Petersburger med. Wochenschr., No. 3, 1887. 
Babuske. Deutsche med. Wochenschr., No. 37, 1888 (recommends phenacetin). 
Day. Headaches, Their Nature, etc. London, Churchill, 1888. 
Nostrom. Cephalalgie et massage. Paris, 1890. 

B. Extracranial Lesions. 

The extracranial lesions are, on account of their great fre- 
quency, of an eminently practical significance and of scientific 
importance, inasmuch as valuable information about physiologi- 
cal questions — that is, the course of the trophic and the gusta- 
tory fibres — may be gained from them if the individual cases 
are carefully observed and accurately recorded. We shall first 
treat of the diseases of the facial branches of the nerve, and 
again separate in our consideration the paralytic from the irri- 
tative affections. 

/. Trigeminal Neuralgia — Fot her gill 's Face-ache — Tic Douloureux 

— Prosopalgia. 

Variable in its degree of intensity, beginning with a moder- 
ate, dull, boring, but always distressing and uncomfortable feel- 
ing of pain, and sometimes reaching a pitch of severity experi- 
enced in no other kind of neuralgia, tic douloureux forms one 
of the most common affections of the fifth nerve. It appears, 
as a rule, unilaterally, and by preference fastens upon the first 
and second branches. The sensory division of the third branch 
seems, at least by itself, rarely to be the seat of the pain, 
whereas it is not uncommon for all three divisions to be simul- 
taneously attacked. 

Almost every patient gives a different description of his 
pain. One declares that it feels as if a red-hot wire was being 
driven into the bone, another as if the face was buried in a 
heap of stinging nettles, a third fancies that the nerves are be- 
ing pierced with a sharp instrument, etc. Almost every case 
presents its own peculiarities as regards the frequency and 
duration of the paroxysms, between which there are often inter- 
vals free from pain, but the course of the disease follows no 
hard and fast rules. The tender points of Valleix {points dou- 
loureux) can almost invariably be demonstrated. Almost al- 
ways there is one on the supra- and another on the infra-orbital 
foramen, a third over the exit of the subcutaneous malae, a 
fourth over the mental foramen, and a fifth in front of the ear, 



THE DISEASES OF THE TRIGEMINAL NERVE. 



69 



where the auriculo-temporal passes over the zygomatic arch. 
The so-called palpebral point on the upper eyelid, the parietal 
point on the parietal eminence, the labial point on the upper 
lip, and many others are not always present. Firm pressure 
on these points is always, even in the intervals between the 
paroxysms, disagreeable to the patient and even liable to pro- 
duce an attack. 

The neuralgia of the first division of the fifth nerve is mostly 
an affection of the supra-orbital nerve, with pain in the fore- 
head, the nose, the upper eyelid, and the eyeball (ciliary 
nerves). The other terminal branches are hardly ever affected. 
The neuralgia of the second division attacks the cheek, the 
lower eyelid, the nose, and the upper lip, often also the upper 
row of the teeth (n. alveol. sup.) and the palate (spheno-palatine 
branch). The branch most commonly affected, sometimes also 
by itself, is the infra-orbital. The neuralgia of the third division 
embraces the lower jaw, the chin, the cheek, sometimes also 
the auricle and the external meatus. The tongue and the mu- 
cous membrane of the mouth may be affected by themselves 
(glossodynia), and this may give rise to the fear on the part of 
the patient that he has cancer or ulceration of the tongue (Lef- 
fers, imagin. ling, ulcerat, Med. News, 1888, xi, 17; cf. also 
Bernhardt, Neurol. Centralbl., 1890, No. 13). Other isolated 
affections of this third division are comparatively rare. The 
only exceptions are the n. buccinatorius, the affection of which 
manifests itself by pain in the anterior part of the ear, which 
radiates to the cheek (Tillaux), and the inferior alveolar branch ; 
the latter is not rarely attacked separately, and the consequent 
toothache has often induced patients to have one tooth after 
another extracted — of course, however, without any improve- 
ment. 

That the vaso-motor and the trophic fibres of the nerves are 
also at times implicated is evident from certain symptoms, viz., 
intense flushing, hyperidrosis, strong pulsation of the temporal 
artery on the diseased side, together with increased secretion 
of tears and saliva, affections of the hair, which has a tendency 
to turn gray and fall out, especially over the most painful 
places. Such symptoms are not uncommon. Herpetic erup- 
tions, especially zoster ophthalmicus and frontalis of the af- 
fected side, have been repeatedly described. 

Pathology. — Of the pathology little can be said with cer- 
tainty. The thickening and swelling of the neurilemma, the 



yo DISEASES OF THE CRANIAL NERVES. 

degeneration of the Gasserian ganglion and of the nerve trunks, 
the small inorganic concretions which have occasionally been 
seen on the neurilemma — all these are changes which have some- 
times been observed, but which quite as often were absent. At 
all events no particular importance can be attributed to them. 
Whether cortical lesions and affections of the nuclei and the 
roots are capable of bringing on the disease we do not definitely 
know, yet such possibilities can not be excluded (cf. the case 
published by myself in the Berl. klin. Wochenschr., 1887, 27). 

The paper of Dana (Journal of Nervous and Mental Dis- 
eases, 1 89 1, xvi, p. 54), in which he claims that disorders in the 
blood-supply, produced by arterio-sclerosis, are often the cause 
of the affection, is interesting ; and it is very desirable that the 
vessels should be carefully examined in such cases. Thoma 
also calls attention to the fact that he has found diffuse arte- 
rio-sclerosis, which was more marked in the neuralgic area 
(Deutsches Arch. f. klin. Med., 1888, xliii, 4, 5). 

Course. — The course of the disease is on the whole extreme- 
ly tedious, and attacks which harass the patient to the end of 
his days are to be observed here as in migraine, the only differ- 
ence being that in the disease under consideration the suffer- 
ings of the patient are still more unbearable. The disease 
throws a shadow over his whole existence far more gloomy 
than in migraine, and so we can well understand why again 
and again he tries all sorts of remedies and frequently even 
the most heroic measures to relieve his pain at a time when a 
migraine patient would have given up all medicine and all 
doctors. 

Treatment. — Unfortunately, here also therapeutics is often 
powerless, as has already been indicated by the remark that the 
disease is often of life-long duration. Hope of recovery is only 
justifiable in cases where we have an underlying disease, as, for 
instance, malaria, in which case the neuralgia is to be regarded 
as a symptom, or where local causes exist — for instance, bone 
diseases, the presence of foreign bodies, or neoplasms which 
can be removed, etc. Such cases will repay the efforts of the 
physician, and a cure can be effected by proper internal medi- 
cation or by surgical interference. In cases, however, where a 
primary cause, which would furnish us with data for our treat- 
ment, can not be discovered — where we, therefore, are forced 
to experiment with the nervines and the so-called specifics — let 
us beware of raising our expectations too high, for too often 



THE DISEASES OF THE TRIGEMINAL NERVE. y l 

all our efforts will be in vain. Arsenic, zinc, quinine, the bro- 
mide and iodide of potassium, asafcetida, castoreum, valerian, 
and many other medicines now completely obsolete have been 
tried, and still to-day sometimes are tried at random. The one 
uses this, the other that drug ; under favorable circumstances 
each one does good once, but rarely is the improvement last- 
ing. Here also the most confidence may be placed in antipy- 
rine and phenacetine, and, if chlorosis be present, in iron (best 
administered in the form of Blaud's pills), quinine, arsenic, and 
iodide of potassium ; if these leave us in the lurch we can re- 
sort to salicylate of sodium, 4.0-6.0 ( 3 j— 3 jss) a day, in cap- 
sules, or to salol or gelsemium, giving the latter in the form of 
the tincture, and pushing it perhaps until slight symptoms of 
intoxication appear (twenty drops ever) 7 two hours). I have 
used corrosive sublimate, 0.05 (five-sixths of a grain a day) in 
pill form, several times successfully. Of butyl chloral I am un- 
able to say anything favorable (butyl chloral hydr., 7.5 (3 jss- 
3ij); glycerin., 20.0 ( § ss.) ; aquae, 130.0 (5iv). Sig. : A table- 
spoonful every ten minutes). In all my cases it proved very 
unsuccessful ; the same holds good for methylene blue, which 
was administered in capsules of 0.1-0.5 and 0.8 pro die (2-7- 
12 grs.). This drug has, besides, disagreeable effects upon the 
urogenital system, giving rise to strangury and pain in the 
glans penis, etc. Other anaesthetics, chloroform above all, do 
at least as much good, and the narcotics are decidedly better, 
as Trousseau has already upheld, who declared large, or we 
should rather say huge, doses of opium or morphine to be the 
only effectual treatment. Whatever we may think about mor- 
phine, in cases of tic douloureux, especially in severe instances, 
we can not dispense with it. The combination of morphine 
with atropine, or the alternate use of the two separately, has 
been recommended (Althaus) ; chloral hydrate alone, 4.0-6.0 
(3 j- 3 jss.) a day, is uncertain in its action, but in combination 
with morphia often acts very well. Cocaine may also be used 
externally or given internally (a teaspoonful of a one-half-per- 
cent solution three times a day). The so-called revulsives 
(daily repeated cold-water enemata (Gussenbauer). cold or 
warm poultices, sinapisms, superficial cauterization, the elec- 
tric brush), often act splendidly where we want to produce 
temporary amelioration of the pain ; but unfortunately this is 
only transient. The same is true of electricity, which may be 
used according to the polar method (steady application of the 



72 



DISEASES OF THE CRANIAL NERVES. 



anode over the painful place, cathode at some indifferent place, 
weak increasing and decreasing- currents being used), or ac- 
cording to the method of direction of the current (descending 
steady current). Ziehl (Berliner klin. Wochenschr., 1889, 12) 
recommends the application of electricity for as long as an 
hour at a time. Galvanism to the neck has also been advised. 
The constant current may be given a trial, combined with the 
action of chloroform, as Adamkiewicz has proposed in his pa- 
per on cataphoresis. I have several times used the " diffusion 
electrode," which he recommends, quite successfully (cf. lit.). 
Hoffmann is also satisfied with the results, but thinks that these 
are not to be attributed to the electricity (Neurol. Centralbl., 
1888, 21). The faradic brush, the unpleasant action of which 
may be somewhat mitigated by putting moist blotting-paper 
on the skin, is often very satisfactory, and I can recommend 
the strong cutaneous faradization very highly even during the 
paroxysms. Operative interference (neurectomy) has recently 
more and more, and justly so, fallen into disrepute. The re- 
sults are often entirely negative, and where some success has 
been obtained with it this did not prove lasting. For an ac- 
count of the method of resection the reader is referred to the 
surgical journals ; the nervus buccinatorius is resected accord- 
ing to the method of Zuckerkandl (Arch. f. klin. Chirurgie, 
1888, 37, 2). In order to avoid relapses it is necessary to con- 
sider the advisability of resecting neighboring nerves (Oba- 
linsky, Wiener klin. Wochenschr., 1889, 41). Repeatedly the 
Gasserian ganglion has been successfully extirpated (Rose, in 
London, Lancet, 1892, x, 22, and Krause, Deutsche med. Woch- 
enschr., 1893, 15). The same is true of the resection of the tri- 
geminus from the foramen ovale (Sulzer, Arch. f. klin Chir., 
1888, 37, 3). Baths, especially at the non-medicated hot springs, 
a stay at the seaside or in the mountains, cold-water treatment, 
and vapor baths may be advised, but we are unable to state 
definitely which of these modes of treatment are indicated in 
any particular case. 

^Etiology. — About the aetiology we know little worthy of 
mention. That heredity and exposure to cold have something 
to do with the disease we must admit ; but this is not peculiar 
to neuralgia of the fifth. However, affections of the pulp of 
the teeth, which are determined by an examination of the sen- 
sitiveness to temperature changes (Boennecken, Berliner klin. 
Wochenschr., 1893, 41), and anatomical changes (exostoses, nar- 



THE DISEASES OF THE TRIGEMINAL NERVE. yo 

rowing of bony canals due to syphilis, etc.), are here frequently 
of moment. Age, sex, and occupation do not seem to exert 
any particular influence ; still, the disease is on the whole very 
rare in small children, and if present it is always inherited. 

LITERATURE. 

Peyrounet de Lafonvlelle. De la neuralgie du trijumeau et en particulier de 
son traitement par les pulverisations de la chlorure de methyle. These de 
Paris, 1886. 

Gussenbauer. Ueber Behandlung der Trigeminusneuralgie. Prag. med. Woch- 
enschr., xi, 31, 1886. 

Schech. Klonische Krampfe des weichen Gaumens mit objectivem Ohrgerausch 
in Folge von nasaler Trigeminusneuralgie. Munchener med. Wochenschr., 
22, 1886. 

Adamkiewicz. Die Diffusionselektrode. Neurol. Centralbl., No. 18, 1886. 

Hirt. Breslauer arztl. Zeitschr., No. 22, 1886. 

Krieger. Centralbl. f. klin. Med., 44, 1886. (Antifebrine.) 

Seifert. Ueber Antifebrin als Nervinum. Wiener med. Wochenschr., 35, 1887. 

Von Frankl-Hochwart. Centralbl. f. d. ges. Therapie, 1888, vi, 9. 

Ziehl. Berliner klin. Wochenschr., 1889, 12. 

Esch. Endresultate der Neurectomie bei Ouintusneuralgien. Inaug.-Dissert., 
Berlin, 1889. 

M. Benedict. Ueber Neuralgie und neuralgische Affectionen und deren Be- 
handlung. Wien, Braumiiller, 1892. 

Hirt. Lehrbuch der Elektrodiagnostik, etc. Stuttgart, Enke, 1893, p. 147 
et seq. 

2. Anczsthesia of the Trigeminus — Paralysis of the Trigeminus. 

Paralysis of the sensory branches of the trigeminus is on 
the whole less frequently met with than neuralgia of the face. 
Only exceptionally are all the divisions (the motor portion of 
the third included) affected equally ; but most observations go 
to show that, as a rule, only individual branches suffer, and 
these not in their whole extent, but only within certain areas. 
The smaller the number of fibres in the distribution of which 
anaesthesia obtains, the more peripheral is the seat of the cause 
(Romberg), and we may assume an affection of one whole 
branch to exist " where the loss of sensation is found not only 
in certain areas of the surface, but also in the corresponding 
cavities of the face " (Romberg). Whether the branch is affect- 
ed before or after leaving the skull we have no means of de- 
ciding. 

A lesion of the first division also causes anaesthesia of the 
surface of the eyeball. Since, in consequence of this, influences 
from outside (foreign bodies, dust, traumatism) are not per- 



74 



DISEASES OF THE CRANIAL NERVES. 



ceived, not infrequently a keratitis, which begins in the lower 
segment of the cornea, is developed. This may run into an 
inflammation of the whole ball and bring about destruction of 
the eye {ophthalmia paralytica). That, to explain this condition 
we must not assume a lesion of special trophic nerves has been 
shown by experiments on animals (Senftleben). An affection 
of the second division deprives the nasal branches of their 
function, and the nose becomes not only insensible to external 
touch, but certain pungent smells — as, for instance, that of 





Fig. 12. — Distribution of the Sensory Cutaneous Nerves on the Head. Fi, V 2y 
V z , the three branches of the trigeminus, at, auriculo-temporal. so, supraorbital, st, 
supratrochlear, it, infratrochlear. /, lachrymal, m, mental, b, buccinator. am y 
auricularis magnus. sm, subcutaneus make, oma and omi, occipitalis major and minor. 
cs, superficial cervical. 

snuff — are no longer recognized on the affected side. In lesions 
of the third division the corresponding half of the tongue, but 
only in its anterior two thirds, loses its sensation, and the pa- 
tient has no longer any sense of taste in this area (n. lingualis) ; 
since, however, loss of taste in the anterior portion of the 
tongue has been observed in some cases where the function of 
the third division of the fifth was found to be perfect (Heus- 
ner), we can not exclude the supposition that the fibres of the 



THE DISEASES OF THE TRIGEMINAL NERVE. jt 

chorda tympani (or at least a considerable part of them) join 
the facial from the second division of the fifth. Certain it is 
that the fibres which pass to the chorda return again to (the 
second and third branch of) the trigeminus after having prob- 
ably run with the facial as far as the geniculate ganglion. 
From Fig. 12 the distribution of anaesthesia over the skin of 
the face may be learned. Vaso-motor changes, subjective sen- 
sations of heat and cold, sensations of swelling, and disturb- 
ances in the movements of mastication and difficulty in open- 
ing the mouth (paresis of the external pterygoid and the ante- 
rior belly of the digastric), are sometimes met with (Miiller). 

The course of the disease depends upon the seat of the 
lesion. In peripheral affections the prospect for recovery is 
usually comparatively favorable ; yet this is frequently only 
partial, and several of the qualities of sensation remain per- 
manently lost, the sensibility in general is dulled, and tactile 
parassthesias persist — in a word, recovery is imperfect. 

The treatment chiefly consists of excitants, among which 
the most efficient is the application of the faradic and the 
galvanic brush to the skin. Transient improvement may be 
thus obtained after a short while in the peripheral affections. 
The electric brush is the best excitant for the skin, and is to 
be preferred to all liniments and the like, which are supposed 
to act in much the same way. Internal treatment, provided 
there be no definite underlying disease, is absolutely super- 
fluous. 

LITERATURE. 

Miiller. Zwei Falle von Trigeminuslahmung. Arch. f. Psych, u. Nervenkrankh., 

xiv, 2, 3, 1883. 
Uthoff. Fall von Neuritis des rechten Trigeminus mit Affection cles Lacrymalis 

und einseitigem Aufhoren der Thranensecretion. Deutsche med. Wochen- 

schr., xii, 19, 1886. 
Dana. A Case of Paralysis of the Trigeminus followed by Alternate Hemiplegia 

— its Relation to the Nerve of Taste. Journ. of Ment. and Nerv. Diseases, 

xiii, 2, p. 65, 1886. 
Heusner. Eine Beobachtung tiber den Verlauf der Geschmacksnerven. Ber- 
liner klin. Wochenschr., xxiii, 44, 1886. 
Ferrier. Lancet, vol. i, No. 1, 1888. 
Ziehl. Virch. Arch., 1889, cxvii, Heft ii (Case of Paralysis of the Third Branch 

of the Trigeminus). 
Ziehl. Ein neuer Fall von isolirter Lahmung des dritten Trigeminusastes mit 

Geschmacksstorung. Virch. Arch., 1892, cxxx, Heft iii. 
Thorbjorn, Hvass. A Case of Anaesthesia of the Trigeminus. Nord. med. Ark., 

1892, new sequence, ii, 6, No. 38. 



j6 DISEASES OF THE CRANIAL NERVES. 

j. Trigeminal Cough. 

Finally, we may call attention to a reflex neurosis, which 
was first described by Schadewald, and then studied by Wille. 
This is a paroxysmal cough which, occurring- in individuals 
whose respiratory organs are perfectly sound, is entirely due 
to an irritation of the trigeminal fibres distributed to the nose, 
pharynx, and the external auditory meatus. These two wri- 
ters distinguish accordingly a nasal, a pharyngeal, and an au- 
ricular trigeminal cough, and declare the first (nasal) to be the 
most frequent variety. According to them also, this neurosis 
is by no means rare, and the possibility of its existence ought 
always to be thought of where we have to treat cases of an 
obstinate paroxysmal cough, which is liable to be produced by 
the action of pungent odors and by changes of temperature, 
and which is accompanied by hypersecretion of the nasal 
mucous membrane. The treatment consists in the use of the 
nasal douche, the application of weak induction currents di- 
rectly to the nasal cavity, and the administration of potassium 
iodide. Further observations are still needed to decide whether 
we actually have to deal in these cases with a neurosis of the 
trigeminus, or whether the vagus has not something to do 
with the affection, or whether, finally, as Hack suggested, the 
erectile tissue of the nose is responsible for it. 

Quite lately it has been claimed that peripheral irritation of 
the trigeminus (by inhalation of pungent vapors, new growths, 
etc.) may reflexly give rise to sensations of dizziness (" nasal ver- 
tigo," Joal). Until more confirmatory evidence is brought for- 
ward, it would be well to suspend judgment on this question. 

LITERATURE. 

Herzog. Der acute und chron. Nasencatarrh mit besonderer Beriicksichtigung 

des nervosen Schnupfens (" Rhinitis vasomotoria"). 2. Aufl., Graz, 1886. 

Leuschner & Lubenski. 
Wille. Der Trigeminushusten. Deutsche med. Wochenschr., xi, 16, ij, 1885. 
Joal. Nasal Vertigo. Lancet, February 1, 1887, p. 31. 
Briigelmann. Ueber Nasenschwindel (aprorexia nasalis). Therapeut. Monats- 

hefte, February, 1889, iii, p. 67. 
Baumgarten, A. Die Neurosen und Reflexneurosen des Nasenrachenraums. 

Volkmann's Sammlung Klin. Vortr., 1892, N. F. 44. 



CHAPTER V. 

DISEASES OF THE FACIAL NERVE. 

The facial nerve emerges at the base of the brain from the 
medulla oblongata by the side of the abducens and behind the tri- 
geminus on the posterior margin of the middle peduncle of the cere- 
bellum. The auditory nerve is situated close behind it, and between 
the two a separate bundle of fibres is placed — namely, a second root 
of the facial, the so-called nervus intermedius or portio intermedia 
Wrisbergii. With the auditory nerve the facial then passes for- 
ward and outward into the internal auditory meatus, at the bottom 
of which it enters through a small opening the Fallopian canal 
(cf. Fig. 15). In the hiatus of this canal it makes an almost rectan- 
gular turn (genu nervi facialis), passes backward and then down- 
ward, and leaves the skull through the stylo-mastoid foramen to 
divide inside of the parotid gland into the terminal branches, the 
temporo-facial and the cervico-facial, which form together the plexus 
anserinus major. At the so-called genu the nerve forms a gangli- 
form swelling — the ganglion geniculi — from which the larger super- 
ficial petrosal nerve is given off (cf. diagram, Fig. 15). These are 
the fibres which communicate with the trigeminus, and have the 
function of gustatory fibres for the anterior two thirds of the tongue 
(cf. page 74). 

The nucleus of the facial, a group of large multipolar ganglionic 
cells, lies four millimetres and a half beneath the floor of the fourth 
ventricle, in the region of the formatio reticularis, dorsal to the 
upper olive (cf. Fig. 13). From this illustration it is also apparent 
that the ascending root of the trigeminus has the emerging portion 
of the facial root to its mesial side, while the anterior root of the 
auditory lies external to it. The axis cylinder processes of the gan- 
glionic cells of the nucleus are united in a larger fasciculus, forming 
the first part of the root (Ursprungsschenkel of Krause), which at 
the floor of the fourth ventricle becomes a compact bundle, the in- 
termediate portion (VII, a). At the anterior end of the eminentia 
teres this is bent at right angles (genu cerebrale), and becomes the 
emerging portion (Austrittsschenkel) of the facial (VII), which 

77 



78 



DISEASES OE THE CRANIAL NERVES. 



reaches its point of exit, before mentioned, through the transverse 
fibres of the pons. 

Quite lately experiments on animals by Mendel have shown that 



n. VI yjL a . 



n.YIIT 




rui 



Fig. 13. — Diagram showing the Course of the Fa- 
cial Fibres in the Pons. (After Schwalbe.) n. VII, 
facial nucleus. VII b, root-bundle of the facial nucleus. 
VII a, intermediate portion (^cross-section). VII, emerg- 
ing portion of the facial, n. VI, abducens nucleus. 
n. VIII, nucleus, and VIII, root of the auditory nerve. 
y, fibres coming from the raphe, x, fibres coming from 
the abducens nucleus, o. s. , upper olive, a. V, ascend- 
ing root of the trigeminus. 

paralysis. We shall discuss each class 



in rabbits and guinea- 
pigs the facial branches 
to the eyes take their 
origin in the oculo-mo- 
tor nucleus. Whether 
this is the case or not in 
man our present patho- 
logical observations do 
not allow us to decide 
with certainty. 

Just as in the case 
of the trigeminus, so 
in the facial, we must, 
distinguish between 
central (cortical and 
bulbar) and peripheral 
(intra- and extra-cra- 
nial) lesions, which, 
owing to the purely 
motor- functions of the 
facial, may give us, 
clinically, spasm or 
separately. 



1. Facial Spasm — " Mimic Facial Spasm 



Tic Convulsif.' 



Lesions which give rise to facial spasm may be central or 
peripheral in their situation. In the first case either the cor- 
tex or the nucleus (or the root) of the nerve in the medulla 
oblongata is concerned. According to our present ideas the 
cortical area for the facial is located in the lower half of the 
anterior and the lower third of the posterior central convolu- 
tions, and it is also supposed that the posterior halves of the 
two lower frontal and the anterior part of the supramarginal 
convolutions have some, although a less important, connection 
with it (Exner). 

It is not known whether stimulation of these centres can 
produce a facial spasm, or, in other words, whether there exists 
a real cortical facial spasm, although the experiences of Cadiot, 



DISEASES OF THE FACIAL NERVE. 



79 



Gilbert, and Roger (Revue de med., May 10, 1890, No. 5) 
seem to leave but little doubt upon this point. It seems, how- 
ever, well established that the disease can be produced by re- 
flex stimulation of the facial nucleus (cf. the case of Berger and 
its treatment). Undoubtedly, disease of the peripheral por- 
tions of the nerve is the most common, in which, just as in tri- 
geminal affections, either the whole facial area or only indi- 
vidual branches may be affected. We distinguish a clonic and 
a tonic variety of spasm. 

A patient suffering from clonic diffuse facial spasm has lost 
control over his facial muscles, either on one or, more rarely, 
on both sides. The muscles affected are in irregular motion, 
so that against his will the patient makes the oddest faces, 
wrinkles his forehead, raises the alse nasi, screws his eyes up, 
etc. When the attack has passed he has a temporary respite, 
yet often enough the pause is very brief, and even during re- 
missions spasms flash across his face, so that his features are 
never for any time entirely at rest. On the slightest provoca- 
tion, by speaking, often also by eating, quite violent paroxysms 
are excited, so that the patient would fain cover up his dis- 
torted face. 

If the spasm is tonic, the affected side of the face is singu- 
larly rigid and takes no part in the facial movements, but is 
distorted. The muscles are distinctly hard to the touch, the 
corner of the mouth is pulled toward the diseased side, the 
mouth firmly closed, the eyebrow drawn up — signs sufficiently 
marked to distinguish it from facial paralysis, in which also the 
affected side does not take part in the movements of expres- 
sion. Vaso-motor and trophic changes are, as a rule, absent. 

In cases where the spasm is confined to some branches of 
the facial only, we find that the muscles around the eyes are 
almost always the ones affected. The eyelid is attacked by a 
clonic or tonic spasm, and conditions are developed which go 
under the names of spasmus nictitans and blepharospasm. 

The spasmus nictitans consists of spasmodic blinking, in 
which not only the eyes are rapidly closed and opened, but 
also the neighboring muscles (frontalis, zygomatici) participate 
in the spasmodic movements. In a mild form this spasm is seen 
in many people where it is only to be regarded as a bad habit. 

Blepharospasm consists of a paroxysmal spastic contraction 
of the orbicularis palpebrarum, lasting a few seconds or min- 
utes, which completely closes the lids. In rare cases the attacks 



80 DISEASES OF THE CRANIAL NERVES. 

follow each other so quickly and are so prolonged that the 
patient has to be treated as a blind man; even a transient 
amaurosis has actually been observed (Silex, Klin. Monatsbl. 
f. Augenheilk., Marz, 1888). The attacks appear unexpectedly 
and quick as lightning. They are often precipitated by volun- 
tary firm closure of the eyes, eye-strain, or by the action of 
light, and the patient is utterly unable to raise the lid until the 
attack has passed. The physician, however, will succeed at 
times in cutting short the paroxysm if he be able to discover 
any of the so-called pressure points, which, according to von 
Graefe, who first discovered them, are often present. More 
or less firm pressure exerted at these points is capable of pro- 
ducing an interruption of the spasm and a cessation of the 
attack. Unfortunately, however, such points are often entirely 
absent, and when they exist their position is so uncertain and 
changeable that they may only be accidentally discovered. 
One of the few which is present with some constancy corre- 
sponds to the supra-orbital foramen. We should, however, 
look for them over the whole distribution of the trigeminus, 
over the spinous and transverse processes of the cervical verte- 
brae, and even in the region of the brachial plexus. It is our 
duty to make a frequent and untiring search for them, as we 
may thus be able to afford our patients very great relief. 

Course. — The course of the disease, be it in the form of a 
total or a partial spasm, is usually very tedious, and a progno- 
sis for recovery must be very guarded. The outlook is espe- 
cially unfavorable when the affection is complicated with other 
motor disturbances, as I have observed, for instance, in two 
cases where the facial spasm was associated with writer's 
cramp. Of late a number of cases have been observed in 
which various motor disturbances were associated with tic 
convulsif ; these conditions have been described as a new dis- 
ease under the name of la maladie des tics convulsifs. We 
shall have occasion to speak of them in our chapter on 
hysteria. 

^Etiology. — We know little about the aetiology of blepharo- 
spasm. That it may be of reflex origin can not be doubted. 
The most varied diseases of the eyes, affections of the nasal 
mucous membrane, or of the trigeminus, especially tic doulou- 
reux, carious teeth, intestinal worms, or uterine troubles, may 
lie at the bottom of it, and the origin of the disease is cleared 
up only if, after removal of some primary cause, the spasm 



DISEASES OF THE FACIAL NERVE. 8 1 

suddenly ceases. An examination of the nose should never be 
neglected. It has repeatedly been noted that the tic disap- 
peared after swellings or tumors of the mucous membrane of 
the nose had been removed (B. Frankel, Peltesohn). 

I saw a case of blepharospasm, which had persisted for 
years and was considered hopeless, cured after a coexisting 
flexion of the uterus had been materially improved. Diseases 
of the blood-vessels can, furthermore, produce the spasm, as 
we see from the case of Buss (cf. lit.), where an atheromatous 
artery, and from that of F. Schultze, where an aneurism of the 
left vertebral artery by pressure upon the facial nerve brought 
on the spasm. Finally, hysterical conditions can lead to it, as 
is shown by the latest communications of Charcot on the so- 
called hemispasmus glosso-labialis, which has been described 
by Marie (Progres med., June 6, 1887). 

Treatment. — All these points we must keep in mind in de- 
ciding upon a line of treatment, and not. imagine that we can 
cure a facial spasm, whether it be total or partial, clonic or 
tonic, with indiscriminate galvanization, for without system we 
shall only meet with success in rare cases, and then only by 
good luck. The most promising plan of treatment is the appli- 
cation of the anode to pressure points if such be present, while 
the cathode is placed on some indifferent region, the back of 
the neck or the sternum. Weak currents applied for one or 
two minutes, with careful avoidance of make and break, give 
the best results. The application of the anode to the back of 
the head, keeping it at the same point, also sometimes meets 
with success (Berger), but too often leaves us in the lurch ; 
and this will hardly surprise us if we remember that even when 
the anode is placed on the back of the neck the abnormally 
stimulated reflex centre in the medulla is by no means always 
reached by those curves of the current which really do pene- 
trate deeply. 

Cures, such as that reported by Berger, undoubtedly de- 
pend upon a happy coincidence of circumstances. The me- 
dulla oblongata, above all, where in such an astonishingly small 
space a number of the most important nuclei lie close together, 
seems to be the most unfavorable place for local electrization 
(Duchenne), by which we aim at affecting individual nerves or 
nerve roots. We may reach all or none, no matter whether we 
use small or large electrodes. Still, even this method ought to 
be tried, since we have no positive remedy. Should the gal- 



82 DISEASES OF THE CRANIAL NERVES. 

vanic treatment fail, the internal treatment is still more vague, 
and it is well to inform the patient of the uncertainty of this 
procedure. Of course, the usual nervines and antispasmodics 
are to be given. Hammond has seen especially favorable re- 
sults from the use of coniin and atropin (Med. Record, No. 41, 
September, 1892). As a last resort, neurectomy of the supra- 
orbital or stretching of the facial nerve (Bernhardt, cf. lit.) has 
to be considered, yet even from this we can expect no lasting 
success. 

LITERATURE. 

Berger. Neurolog. Centralbl., 10, 1883. 

Bernhardt. Arch. f. Psych, und Nervenkrankh., xv, 3, 1884. 

Buss. Neurol. Centralbl., 14, 1886. 

Henschen. Reumatick tic convulsif med fortjocking af nervi facialis stam. Up- 

sala lakareforen. fbrh. 1887, xxiii, 3. 
Guinon. Tics convulsifs et hysteric Revue de m6d., June, 1877. 
Cf. besides the text-books of Striimpell, Seeligmuller, Eichhorst, Eulenbu.rg, etc. 

2. Facial Paralysis — Mimic Facial Paralysis — Hemi- 
plegia Facialis — Prosopoplegia. 

Facial paralysis is an affection the relative frequency of 
which makes it of the greatest practical importance. In this 
more than in any other nervous disease any layman can easily 
judge just how much the art of the physician has accomplished 
in a certain time in a given case, and on this account it espe- 
cially behooves us at our first examination to be very careful 
in making a positive statement as to the prospects of recovery 
or the probable duration of the disease. Both these points de- 
pend chiefly on the seat of the lesion, which, as in tic convulsif, 
may be central or peripheral. 

A. CENTRAL FACIAL PARALYSIS. 

Symptoms and Diagnosis. — Central facial paralysis may be 
produced either by a cortical lesion (cortical paralysis) or by a 
lesion of the facial fibres in the brain between the cortex and 
the pons (intracerebral paralysis par excellence) ; or, finally, it 
may depend upon a disease of the nuclei and nerve roots in the 
pons. Cortical facial palsies may be caused by tumors, ab- 
scesses, or chronic inflammations in the region of the motor 
centres. Those of intracerebral origin may be produced by 
syphilitic arterial disease or by rupture of a vessel in the 
region of the internal capsule and the crus cerebri. The third 



DISEASES OF THE FACIAL NERVE. 



83 



form, that originating in the pons, is found in Duchenne's 
paralysis and, more rarely, in tabes. There exists a form of 
facial paralysis the pathology and the seat of which is as yet 
very obscure, and we can only say that probably a " nervous 
predisposition " is necessary for its development. It may oc- 
cur in several members of the same family, may be congenital, 
and may be associated with paralysis in the region of the sixth 
nerve and of the trigeminus. It sets in without any appre- 
ciable cause, is wont to recur, and may last for an indefinite 
period of time ; probably its anatomical seat is in the nucleus 
(" infantile degeneration of the nucleus," Mobius), but, as was 
said above, this is by no means proved. It is quite possible 
that some cases may have a peripheral origin, as is the case in 
the recurrent oculo-motor paralysis (cf. page 50). 

The clinical picture differs but little in these three forms, 
and only at times do the accompanying symptoms make a dif- 
ferential diagnosis possible. Thus, for instance, the intracere- 
bral paralysis often appears with an apoplectic attack, and is 
accompanied by hemiplegia and speech disturbances, while if 
facial paralysis is found in connection with spinal disease it is 
always of nuclear origin. 

All three forms of central paralysis have usually, however, 
two features in common which can almost be regarded as 
pathognomonic and which distinguish them from the periph- 
eral paralysis, namely: 1. The presence of a normal electrical 
excitability in the nerves and muscles to both currents. 2. The 
escape of the upper facial branch. While in peripheral paraly- 
sis all three divisions are equally affected, we find in the cen- 
tral form the upper branch usually intact, and the patient can 
wrinkle his forehead and close both eyes. 

We say usually, not always, because there are undoubtedly 
exceptions, where we meet with a central paralysis in which the 
upper branch has not been spared. It is quite probable that 
the naso-labial and the orbiculo-frontal fibres of the facial have 
a separate cortical origin, and we can well imagine that if the 
cause of the paralysis — e. g., a small focus in the cerebrum — is 
situated above the union of those two branches, one remains 
intact (in the large majority of cases the upper), whereas if it is 
below their point of union both branches are affected. 

A further guide to localization is the condition of the move- 
ments of expression (Bechterew). If these are lost while the 
voluntary innervation of the facial muscles is intact, we have to 



8 4 



DISEASES OF THE CRANIAL NERVES. 



assume a focus in the optic thalamus, the centre for facial ex- 
pressions, or close to it (Bechterew), while a facial paralysis 
with retained power of facial expression allows us to exclude a 
lesion in the thalamus and its coronal connection with the hemi- 
spheres. In the case of Rosenbach (Neurol. Centralblatt, n, 
1 886) there was an isolated paralysis of mimic expression in the 
left facial and right-sided bilateral hemianopia, and the lesion 
was taken to be in the right thalamus. 

In differentiating between a cortical and a bulbar facial pa- 
ralysis the following points must be taken into consideration : 
That the lesion is cortical is probable if the facial alone with- 
out the corresponding half of the body 
is paralyzed (monoplegia facialis), and 
if the affection is confined to the lower 
branches of the nerve, while the nor- 
mal reaction to the electrical current 
remains undisturbed. It is easy to 
understand that the hypoglossus often 
takes part in the lesion if we remember 
in how close proximity the centres of 
the two nerves are situated in the cor- 
tex, and in a given case an examination 
of the mobility of the tongue will show 
whether we actually have to deal with 
a so-called monoplegia facio-lingualis. 
Sometimes a disturbance of speech 
points at once to this combination. In 
every case in which we assume a cor- 
tical lesion, the sensation in the distri- 
bution of the facial and the hypoglossus 
ought to be tested, because it is just in 
these cases that we find not infrequently 
sensory changes — e. g., analgesias and 
anaesthesias. 

We shall be led in a facial paraly- 
sis to think of an affection of the pons 
when not only the nerve, but with it 
one whole half of the body is para- 
lyzed ; and there are two types of pon- 
tine facial paralysis according as the lesion is situated in the 
upper or lower part of the pons. In the first case (focus a in 
Fig. 14) the facial and the same, in the second (focus b) the 




Fig. 14.— Diagram showing 
the Decussation of the 
Fibres going to the 
Extremities, and of 
those going to the 
Face, in the Pons and 
Medulla Oblongata. F, 
facial fibres. F, fibres go- 
ing to the extremities. P, 
pons. O, medulla oblon- 
gata, pyx, decussation of 
the pyramidal tracts. #, a 
focus in the upper, &, a 
focus in the lower, part of 
the pons (the latter is situ- 
ated below the decussation 
of the facial fibres). 



DISEASES OF THE FACIAL NERVE. 



85 



facial and the opposite half of the body are affected (hemiplegia 
alternans, Gubler, 1859), because the facial fibres cross in the 
pons and we may have a lesion above or below this crossing, 
and in both cases this will be situated, of course, above the 
crossing of the fibres going to the extremities. 

The facial paralysis caused by the lesion in the upper part 
of the pons, and that found in connection with hemiplegia after 
a lesion in the internal capsule, are in so far alike as they are 
both accompanied by paralysis of the extremities on the same 
side. But there is one point of difference which will influence 
our diagnosis, namely, that after pontine lesions the facial pa- 
ralysis, very much as in the peripheral form, takes in all three 
branches of the nerve, while in a lesion of the capsule or the 
basal ganglia only the lower branches of the nerve are affected ; 
but in contradistinction to what happens in the peripheral 
paralysis the electrical condition may, at least in some cases, 
remain normal. 

The most striking symptom of central facial paralysis is the 
relaxed and expressionless appearance of the affected side. 
The naso-labial fold is more or less distinctly flattened, the cor- 
ner of the mouth is slightly open and hangs down, the mouth 
seems to be drawn to the well side, the patient is unable to 
raise his upper lip or to whistle. On inflating the cheeks the 
air escapes ; drinking and speaking are difficult, the latter espe- 
cially, because the labial sounds are defectively formed. Dur- 
ing eating the food gets in between the cheek and the teeth on 
the affected side, and the patient has to bring it to the right 
place again with the fingers. In biting, the mucous membrane 
of the cheek is often caught between the teeth. The upper part 
of the face is in by far the greater number of cases normal ; the 
forehead can be wrinkled well in its whole extent, and the pa- 
tient can frown and close either eye perfectly. 

The condition of the velum palati and the uvula varies, and 
is, therefore, of no value, either diagnostically or prognostically. 
The uvula may deviate to the sound or to the affected side, 
or may occupy its normal position. With our still imperfect 
knowledge of the innervation of the muscles concerned, any 
attempt to explain the different positions of the uvula must 
needs be hard, but we shall be less surprised at our difficulty 
when we consider that the levator palati is supplied not only 
by the facial through the large superficial petrosal, but very 
probably also by the vagus accessory, the tensor palati, how- 



86 DISEASES OF THE CRANIAL NERVES. 

ever, by the third branch of the fifth. In other words, at least 
three cranial nerves are concerned in the motion and fixation 
of the uvula, and besides, even under normal conditions, the 
uvula is occasionally found to deviate to one or the other side. 
The only thing of which we can, perhaps, be sure is that if 
during phonation paresis of the velum palati and deviation to 
the sound side becomes apparent, the large superficial petrosal 
is most likely affected (paralysis of the levator palati and azj'gos 
uvulae). Of greater importance for the diagnosis of central facial 
paralysis is the persistence of the reflexes, which in peripheral 
paralysis are often diminished or sometimes completely lost. 

Furthermore, the disturbances in hearing, the alterations in 
taste and in the salivary secretion, so frequently observed in the 
latter, are almost always absent in central affections. 

The existence of a bilateral facial paralysis- — diplegia facialis 
— points as a rule to a central lesion, and more especially to a 
bulbar affection. It certainly is one of the greatest rarities to 
have a simultaneous paralysis of both as the result of a periph- 
eral lesion. 

Prognosis. — The prognosis depends upon the anatomical 
basis of the disease. Lesions of the cortex and the pons often 
bring about facial paralyses that are incurable, while those ob- 
served in conjunction with capsular hemiplegias, especially in 
the early stages of the latter, frequently present a decided im- 
provement after a time. As was stated above, it is impossible 
in the cases which depend upon a neuropathic predisposition 
to make any statement either with reference to duration or with 
reference to a possible recurrence of the trouble. 

Treatment. — The question of treatment arises only when 
the primary lesion is amenable to therapeutic measures. Since 
this, however, is only very rarely the case, it is best, at least in 
the central facial paralysis, to restrict ourselves to the expect- 
ant treatment. The measures that will be recommended as in- 
dicated in the peripheral form are here of very little avail. 

B. PERIPHERAL FACIAL PARALYSIS. 

In its peripheral course the facial may be divided into two 
portions — an intracranial and an extracranial. The former is 
less frequently affected than the latter, which is more ex- 
posed to atmospheric influences, especially cold. For prac- 
tical reasons we prefer to consider the diseases of these two 
segments separately. 



DISEASES OF THE FACIAL NERVE. 



87 



I. The Intracranial Lesion. 

This form is distinguished by the fact that besides the con- 
stant existence of paralysis of all the facial branches, we have 




Fig. 15. — Erb's Diagram for Facial Paralysis. Representing the course of the facial 
trunk from the base of the skull to the pes anserinus. N. a., auditory nerve. N. /., 
facial nerve. N. p. s., large superficial petrosal nerve. G. g., geniculate ganglion, 
N. c. c. p. t. y communicating branch to tympanic plexus. N. st., stapedius nerve. 
Ch. t., chorda tympani. G. f., gustatory fibres. Sps., secretory nerve to salivary 
glands. F. st., stylo-mastoid foramen. N. a. p., posterior auricular nerve. 

often certain concomitant symptoms, which can only be fully 
understood if we picture to ourselves the exact course of the 



88 DISEASES OF THE CRANIAL NERVES. 

nerve. This can be done with the help of the diagram (taken 
from Erb) here represented, which permits an accurate locali- 
zation of any given intracranial lesion. 

(a) If the lesion be between the exit of the facial stem (from 
the pons) and the geniculate ganglion, we shall find a paralysis 
of the velum palati, abnormal acuteness of hearing, and dimin- 
ished salivary secretion. 

(b) If the facial be affected in the region of the geniculate 
ganglion itself, then we find in addition to the just-mentioned 
symptoms alterations in the sense of taste. 

(c) A lesion between the geniculate ganglion and the stape- 
dius nerve produces the symptoms described in a and b, but no 
abnormality of the velum palati. 

(d) A lesion between the origin of the nerve to the stape- 
dius muscle and the giving off of the chorda tympani gives 
alterations in the sense of taste and diminishes salivary secre- 
tion, but no abnormality of hearing or of the velum palati. 

(e) If, finally, the nerve is diseased below the giving off of 
the chorda, in the Fallopian canal, we only find paralysis in the 
distribution of the posterior auricular branch without any 
trouble with taste, hearing, the condition of the velum palati, 
or the secretion of saliva. We should state again, however, 
that in all cases from a to e all the facial branches take part in 
the paralysis. 

Valuable as this diagram is, undoubtedly, regarded from a 
theoretical stand-point, yet in practice we but rarely meet with 
opportunities for observing cases which exactly correspond to 
it ; nevertheless, in every instance we should not fail to attempt 
to locate the lesion with as much accuracy as possible. 

A physiological explanation for the appearance of the above- 
mentioned concomitant symptoms is not always easy. That 
alterations in the sense of taste are due to lesions of the chorda 
tympani can not be doubted, and if they are present the lesion 
is situated between the geniculate ganglion and the giving off 
of the chorda ; if they are absent the lesion must be sought 
below this region. The disturbance in the sense of taste is 
limited to the anterior two thirds of the tongue, and exists, of 
course, only on the paralyzed side. Sensory changes in the 
tongue are not necessarily present. Less clear is the cause of 
the diminished salivary secretion. Its occurrence is said to 
point to a lesion above the geniculate ganglion (Wachsmuth). 
Mendel has observed increased salivary secretion in an instance 



DISEASES OF THE FACIAL NERVE. 



8 9 



in which it was also difficult to find an adequate physiological 
explanation (Neurol. Centralblatt, 1890, 16). 

Among the most common and best known symptoms are the 
disturbances in hearing, which consist either in an abnormal 
acuteness of hearing (hyperacusis, oxyacoia) or in a decrease 
in the power of hearing. In the first case, where we have a 
kind of hyperesthesia for all musical tones, the alteration is 
supposed to be due to a paralysis of the stapedius muscle (which 
is supplied by the facial) and a consequent overaction of the 
tensor tympani (Lucae, Hitzig, Roux). The latter — the hard- 
ness of hearing — can be due to several causes. We may either 
have a disease of the middle ear and the adjoining portion of 
the temporal bone, which has affected the facial nerve by con- 
tiguity, or a simultaneous affection of the auditory nerve, which, 
in the internal auditory meatus, has been exposed to the same 
deleterious influence, and become affected by the same disease 
as the facial. Quite lately again the frequency of this combina- 
tion of facial paralysis with a slight paralysis of the auditory 
nerve has been pointed out by O. Rosenbach (cf. lit.). 

2. The Extracranial Lesion. 

The peripheral paralysis of the facial after its exit from the 
skull is, as we have already said, the most common. Of this 
class the so-called rheumatic form, which is attributed to the 
influence of cold ( a f rigor e), and the traumatic, often observed 
after operations, gunshot injuries, etc., or which may be caused 
by the pressure of impacted cerumen in the ear and mastoid 
cells (Dalbey, New York Med. Journal, liv. 3, 1891), are the 
two chief representatives. When any one, heated as he is, 
passes from a warm room into a cold wintry night, or is ex- 
posed to draughts in the railroad cars, and finds himself a few 
hours later taken with a paralysis of one side of the face, this 
is the so-called rheumatic form which has attacked the stem of 
the nerve after its exit from the Fallopian canal. But the influ- 
ence of cold in such instances must be regarded only as the 
precipitating cause in individuals with a neuropathic predis- 
position (Neumann, Arch. d. Neurologie, July, 1887, xiv, 40). 

In these cases all three facial branches are affected, and the 
appearance of the patient is changed in a very material and 
striking manner. Even the layman notices that the patient 
now wrinkles only one half of his forehead, and that the folds 
and furrows generally present are obliterated on one side ; that 



9 o 



DISEASES OF THE CRANIAL NERVES. 



he can shut one eye only while the other remains wide open and 
can not be closed despite the strongest efforts. If the attempt 
is made, the eyelids remain gaping, the eyeball is rolled inward 
and upward, and the pupil disappears behind the upper lid, a 
position which is also maintained during sleep (lagophthalmos). 
The inability to shut the lids prevents the tears from running 
into the tear ducts and interferes with the process by which 
foreign bodies, particles of dust and the like, are removed from 
the eye. It happens, then, that the tears are always running 
down the cheeks, and that a conjunctivitis, even an ulceration 
of the cornea, may be developed through the mechanical irri- 
tation caused by such foreign bodies. The appearance of the 
lower part of the face has already been described. In mild 
cases the tongue does not deviate at all ; in grave cases it is 
turned toward the well side (Hitzig, cf. lit.). 

It is interesting to note that in the first stage of rheumatic 
facial paralysis the patient often complains of pains the inten- 
sity of which seems to be proportional to the degree of the 
paralysis. These are usually localized in front or behind the 
ear and radiate toward the forehead, the temple, and the cheek ; 
sometimes they last but a few days, in other cases they persist 
for weeks. They must be referred to an affection of sensory 
branches belonging to the trigeminus. 

The hyperidrosis associated with facial paralysis, as ob- 
served by Windscheid (Miinchener med. Wochenschr., xxxvii, 
50, 1890), as well as the frequently noted puffiness and the porce- 
lain-like induration of the affected side associated with vascular 
dilatation and elevation of temperature (von Frankl Hochwart, 
Deutsch. med. Ztg., 1891, 35), show that vaso-motor fibres are 
also implicated in facial paralysis. I have observed the appear- 
ance of cedematous swelling especially in the recurrent forms. 
The implication of trophic fibres is shown by the not rare oc- 
currence of herpes zoster, which has recently been described 
by Letulle, Striibing, Voigt, and Perrin (cf. lit.). Whether 
this is due to an inflammation of the peripheral endings of the 
fifth, which is transmitted to the facial (Striibing), or whether 
the stem of the facial contains in parts fibres, an inflammatory 
irritation of which may produce herpes zoster (Eulenburg), is 
not clear. 

I have only in rare instances seen this complication, and 
have found that whenever it was present the cases pursued an 
unusually protracted course. 



DISEASES OF THE FACIAL NERVE. 



91 



Duration and Course. — The duration and course of rheu- 
matic facial paralysis are extremely variable, and it is of great 
importance for the physician to be able to give at the begin- 
ning an approximately accurate opinion as to the length of time 
necessary for recovery. This we can, however, only do if we 
investigate the electrical condition of the paralyzed muscles, 
and hence it follows that it should be our invariable rule to 
make an electrical examination before venturing upon any 
expression of opinion. The following are the chief points to 
guide us : 

1. If we find no changes either in faradic or in galvanic ex- 
citability the prognosis is favorable ; recovery in from seven to 
twenty days (light form). 

2. If we find the faradic and galvanic excitability of the 
nerve diminished, but not lost, the galvanic excitability of the 
muscles, however, increased, and the usual formula of contrac- 
tions changed (A. C. C. > C. C. C), then the prognosis is rela- 
tively favorable ; recovery in from four to six weeks (interme- 
diate form of Erb). 

3. If the reaction of degeneration be found — i. e., if the far- 
adic and galvanic excitability of the nerve and the faradic ex- 
citability of the muscles be lost, while there is an increase in 
the galvanic excitability of the muscles associated with quali- 
tative changes and changes in the mechanical excitability — then 
the prognosis is relatively unfavorable, and for recovery two, 
four, six, eight, even twelve months, may be required (grave 
form). These are those bad cases in which secondary con- 
tractures and spasmodic twitchings of the muscles also appear, 
which, according to Hitzig's opinion, are to be referred to an 
obscure abnormal irritation in the medulla oblongata. It is 
well to know that, as convalescence begins, voluntary motion 
may return long before the electrical excitability, so that often 
the patient is able to perform some slight voluntary movements 
before faradic stimulation provokes the least contraction. 

The pathological changes have been studied by Minkowski 
(Berliner klin. Wochenschrift, 1891, 27), and quite recently by 
Darkschewitsch and Tichonow (Neurol. Centralblatt, 1893, 10). 
The latter found a parenchymatous neuritis in the peripheral 
portion of the nerve, and in the central portion the signs of 
secondary degeneration, with many perfectly atrophied fibres ; 
in the nuclei also the signs of a well-marked atrophy were 
present. 



9 2 



DISEASES OF THE CRANIAL NERVES. 



Diagnosis. — With regard to the diagnosis there is even for 
the beginner no more easily recognizable disease. Still, there 
are cases where it is difficult, not to say whether there is any 
paralysis, but, strange as it may sound, which is tne affected 
side. One is particularly liable to mistakes in old people, in 
whom the wrinkled, inelastic skin has produced a stereotyped 
expression, which, even when the facial muscles contract, is 
but little changed. Suppose now the muscles to have lost their 
innervation, the paralyzed side takes on the soft features of an 
earlier period of life, and this may go so far that the patient 
believes his rigid, wrinkled side to be the paralyzed, and the 
affected side the healthy one (Gowers). We also must remem- 
ber that the non-paralyzed zygomatici pull the face sharply 
toward the well side, a condition which easily produces in the 
layman the impression of something abnormal, so that he takes 
the side thus distorted for the diseased one. In general, how- 
ever, we may say that the diagnosis of a peripheral facial pa- 
ralysis is one of the easiest imaginable in neuropathology. 

Treatment. — In the treatment we may in recent cases rec- 
ommend for trial steam-baths and counter-irritation to the skin ; 
but never, unless there is a special indication, should internal 
remedies be advised, because in a non-complicated rheumatic 
facial paralysis they are absolutely superfluous. In more pro- 
tracted cases the methodical use of electricity is strongly indi- 
cated, for even though it is undoubtedly true that the disease, 
if the prognosis is at all favorable, gets well of its own accord, 
and really requires no treatment at all, there can, on the other 
hand, be no doubt but that the electrical treatment hastens the 
cure in a marked degree ; therefore, electricity should be used 
under all circumstances. Just which method should be em- 
ployed can not be definitely laid down, but we should keep in 
mind that not only the galvanic current is beneficial, but that 
the faradic brush applied to the stem and the individual 
branches of the facial gives good results, and the patient 
should, therefore, be persuaded to submit to this somewhat 
disagreeable procedure. The places from which the most im- 
portant facial muscles can best be stimulated are seen in Fig. 
16. At these points the motor-nerve branches to the muscles 
concerned lie very near the surface. They are called " motor 
points " (Ziemssen). In galvanization every specialist has his 
pet method of application and his own ideas about the strength 
and direction of the current. The one prefers to apply the 



DISEASES OF THE FACIAL NERVE. 



93 



electrode over the mastoid process, placing either the anode or 
the cathode on the affected side of the face ; another treats at 
the same time the sympathetic in the neck; a third, again, ap- 
plies the anode over the affected nerve and the cathode to an 
indifferent point, and so forth. Whichever method we may 



Lower branch of 
the facial. 



Region of the cen- 
tral convolutions. 



Region of the 
is^ speech centre. 



Upper branch of 
the facial. 



Facial stem. 
Middle branch of the facial. 




N. accessorius. 



Supraclavicular 
point (plex. 
brach.). 



Fig. 16. — Some of the so-called "Motor Points" on the Face and Neck. 

prefer, the main thing, after all, is to produce by repeated 
opening and closing of the current contractions of the mus- 
cles by which the tonus of the latter will soon be improved. 
I should like to mention, too, that I have seen the application 
of the galvanic brush and the use of the combined current (de 
Wattewille) repeatedly attended with satisfactory results (Hirt, 
Lehrbuch, etc., loc. cit., p. 102 et seq.). 



LITERATURE. 

Romberg. Op. cit., pp. 349 et seq. 
Letulle. Arch, de Physiol., 2me ser., ix, p. 662, 1882. 

Eulenburg. Ueber Complicationen von peripherer Facialislahmung mit Zoster 
faciei. Centralbl. f. Nervenheilk., 5, 1885. 



94 DISEASES OF THE CRANIAL NERVES. 

Voigt. Petersburger med. Wochenschr., ix, 45, 1885. 

Remak. Centralbl. f. Nervenheilk., 5, 1885. 

Striibing. Deutsches Arch. f. klin. Med., xxxvii, p. 513, 1885. 

Dargaud. De l'hemiplegie faciale dans la periode secondaire de la syphilis. 

These de Paris, 1885, No. 178. 
Mobius. Centralbl. f. Nervenheilk., ix, 7, 1885. 

Dana and Wilkin. Journ. of Mental and Nerv. Diseases, No. 7, 1886. 
Eichhorst. Handbuch der speciellen Pathologie und Therapie, Bd. iii, 3. Aufl. 

Wien, 1887. 
Chisolm. Arch. f. Augenheilk., xvii, 4, p. 414, 1887. (Congenital Paralysis of 

the Sixth and Seventh Pair of Cranial Nerves.) 
Mendel. Ueber den Kernursprung des Augenfacialis. Neurol. Centralbl., No.. 

23, 1887. 
Huet. Hysterical Facial Paralysis. Weekblad v. d. Nederl. Tijdschr. v. 

Geneesk., 25, 1887. 
Mobius. Ueber die angeborene doppelseitige Abducens-Facialislahmung. 

Miinchener med. Wochenschr., 1888, 86. 
Roulland. A propos de quelques faits de paralysies des nouveau-nes. Paris, 

Steinheil, 1889. 
Foucher. De la contracture secondaire des muscles de la face. Th&se de 

Paris, 1888. 
Stephan. De la paralysie faciale des nouveau-nes. Revue de med., 1888, 7. 
Demoulin. De la paralysie faciale tardive dans les fractures du rocher. Gaz. 

med. de Paris, July 14, 1888. 
Vizioli. On the ^Etiology of Facial Paralysis a f rigor e. Riforma med., 1888, 

pp. 279, 280. 
Bernhardt. Ueber angeborene einseitige Trigeminus-, Abducens-, Facialis- 

lahmung. Neurol. Centralblatt, 1890, No. 14; Ueber Facialislahmung und 

Facialiskrampf. Berliner klin. Wochenschr., 1892, xxix, No. 51. 
Perrin. Journ. des malad. cut. et syph., November, 1892. 
Decoux. De la paralyse faciale hysterique. Gaz. de Paris, 1892, 47. 
Hitzig. Die Stellung der Zunge bei peripherer Lahmung des Facialis. Berliner 

klin. Wochenschr., 1892, 50. 
Darkschewitsch und Tichonow. Pathological Alterations in Peripheral Facial 

Paralysis. Med. Obosr., 1892, 18 (Russian). 
Stintzing. Ueber Diplegia facialis (Prosopodiplegia). Miinchener med. Woch- 

enschr., 1893, 1. 



CHAPTER VI. 

DISEASES OF THE AUDITORY NERVE. 

The auditory nerve emerges at the base of the brain, alongside 
of the facial, and takes with this latter a forward and outward course. 
After having entered the internal auditory meatus, it divides before 
reaching the cribriform plate, which separates the internal meatus 
from the internal ear, into two main branches, an anterior inferior 
and a posterior superior. These nerves pass as small filaments 
through the openings in the plate, to be distributed respectively to 
the cochlea and vestibule, and are hence called ramus cochlearis and 
ramus vestibularis. 

The cortical centre of the nerve is probably to be sought for in 
the temporal lobe ; the fibres are said to run through the last third 
of the posterior division of the internal capsule, through the middle 
geniculate body, through the brachia conjunctiva posteriora, the 
posterior corpora quadrigemina, and the inferior fillet (v. Monakow, 
Baginsky). 

About the situation of the nuclei of the auditory nerve there 
seems still to exist a difference of opinion among the anatomists. 
Usually two nuclei are distinguished, an inner or principal nucleus 
and an outer one situated laterally from the first. In their structure 
these present material differences. While the former — the inner 
nucleus — only contains scattered, small, slender, ganglionic cells (15 
to 20 fA long), the latter contains cells of considerable size (60 to 100 
fz long and 15 to 21 //, broad). The situation of the two nuclei may 
be understood from the accompanying diagram. 

Of the two roots, the superficial terminates in the internal audi- 
tory nucleus, while the deeper one passes between the restiform body 
and the ascending root of the fifth, and turns toward the outer one. 
This, also, the diagram, which is taken from Wernicke, and which 
demonstrates the views of Meynert, illustrates. 

Although the diseases of the auditory nerve are not, as a 
rule, treated of in neurological text-books, they are found some- 
times so closely connected with other nervous diseases, and 

95 



9 6 



DISEASES OF THE CRANIAL NERVES. 



are, notwithstanding their comparative rarity, of such decided 
practical importance, that we feel not only justified but com- 
pelled to consider them here, at least briefly. 

The nerve, as we have said, rarely ever becomes primarily 
diseased, but diseases of the middle and internal ear — that is, 
secondary affections — are by far the most common causes of 




Fig. 17.— Diagrammatic Section through the Medulla Oblongata in the Region 
of the (Lower) Olive. The right half represents a lower plane, p, pyramids ; oz\ 
lower olive ; /, lemniscus ; mf, motor region of the tegmentum ; 5, ascending root of the 
fifth; «'., corp. restif. ; 8.e., external, 8./., internal nucleus of the auditory ; et., emin. 
teres; 12, nucleus and root of the hypoglossus ; 10, root of the vagus; X.a., anterior, 
X. p., posterior vagus nucleus ; X./., combined root of the " lateral mixed system " (cf. 
p. 105) ; 8.s., superficial, 8./., deep root of the auditory nerve. 

diminution or loss of hearing. We may distinguish between 
conditions of irritation and those of paralysis, so that on the 
one hand we shall have hyperaesthesias, on the other pareses 
or paralyses. 



I. Hyperesthesias of the Auditory Nerve. 

We speak of a hyperaesthesia of the auditory nerve where 
the patient experiences a painful sensation in his ear when per- 
ceiving certain sounds or noises. For instance, in excitable 
and nervous individuals who suffer from hemicrania or tic 
douloureux, such a sensation may be produced by high musical 



DISEASES OF THE AUDITORY NERVE. 



97 



notes, whistling, and the like. Quite a different affection is an 
abnormal acuteness of hearing, which is extremely rare, the so- 
called oxyacoia of which we have spoken in the chapter on 
facial paralysis. 

Frequently one hears nervous patients complaining of sub- 
jective auditory perceptions, roaring, buzzing, hissing, singing, 
humming, and the so-called nervous tinnitus aurium, which 
may persist during the whole life without a sign of any other 
disturbances of function. This symptom may be due to a 
purely functional disorder or it may be the forerunner of a 
middle-ear sclerosis. 

Therapeutically, we may, after the removal of masses of 
cerumen or epidermis which may have obstructed the outer 
canal, with benefit make use of blisters, stimulating lotions ap- 
plied to the mastoid process, subcutaneous injections of mor- 
phine, the bromides, digitalis, and atropine. If abnormalities 
of tension in the sound-conducting apparatus and consequent 
rise of pressure in the labyrinth be the cause of the disorder, 
then the inflation of the middle ear and the rarefaction of the 
air in the outer canal is to be recommended. 

II. The Pareses and Paralyses of the Auditory Nerve. 

Analogous to the rheumatic facial paralysis we have a con- 
dition in the auditory nerve which manifests itself in either a 
decrease or a loss of the function of hearing, the so-called rheu- 
matic acusticus paralysis. It is less frequent than the former, 
although the cause of both, namely, cold, is the same. Central 
paralyses are always connected with decrease of hearing power 
on one side only. Absolute unilateral deafness, as a conse- 
quence of a focal lesion in one of the hemispheres, has up till 
now not been observed (Wernicke). Whether the disturbances 
in hearing observed by Baginsky in railway spine are of a cen- 
tral or peripheral nature remains yet to be studied (cf. lit.). 

Next in order we have to mention in this connection the 
anaesthesia and paresis of the auditory nerve, which sometimes 
appear quite suddenly in the course of hysteria, and often as 
suddenly disappear again after a longer or shorter period of 
time. 

Of interest from a pathological standpoint is the nervous 
deafness occurring after an epidemic cerebro-spinal meningitis. 
It is this form which has been so thoroughly studied by Moos. 



98 DISEASES OF THE CRANIAL NERVES. 

There is hardly any doubt but that it is caused by the passage 
of purulent masses from the meninges along the sheath of the 
auditory nerve into the inner ear. The prognosis is unfavor- 
able. A diminution of hearing, probably due to transitory cir- 
culatory disturbances, occurs sometimes after epileptic attacks. 
Although not common, this affection is certainly well authen- 
ticated. 

Of especial interest serologically are the disorders of hear- 
ing which we find in engineers and firemen on the railroads as 
a consequence of their occupation. This must principally be 
attributed to the noise, aided, however, to some extent by the 
abrupt and severe changes of temperature and the exposure to 
all kinds of weather. We do not know anything positive about 
the relative frequency of this affection, which consists in a more 
or less pronounced decrease of hearing, but in the general in- 
terest of the public it certainly deserves as much attention on 
the part of the companies as the color-blindness which has for 
years been carefully looked into. Locksmiths, blacksmiths, 
and boiler-makers, whose auditory nerves are also being con- 
stantly overstimulated, suffer from similar disorders. In rare 
and exceptional cases it has been observed that mechanics who 
are " hard of hearing " hear better during the usual noise con- 
nected with their work than when everything around them is 
quiet — paracusis Willisii. This very remarkable phenomenon 
is probably due to a decrease in the vibratory power of the 
auditory ossicles, owing to which the sound is conducted with 
more difficulty, a condition which is obviated by a more forci- 
ble concussion (Buerkner, Roosa). We would not leave un- 
mentioned the fact that an overtaxation of the auditory nerves 
lasting for years causes great nervousness, and may even pre- 
dispose to mental diseases. 

In the treatment, endermic inunctions of strychnine (o.i to 
glycerin io.o (gr. jss. ; glycerin, 3 ijss.) — sig., ten drops) over 
the mastoid process, and fumes of sulphuric ether conducted by 
a catheter into the tympanic cavity to act on the distribution of 
the acusticus, deserve recommendation. A beneficial effect 
from the galvanic current can be expected only if examination 
assures us that the current has a modifying influence on the 
subjective noises or upon the power of hearing. This treat- 
ment necessitates a knowledge of the investigations of Brenner 
on the galvanic reactions of the auditory nerve (cf. Hirt, loc. 
cit., p. 109). 



DISEASES OF THE AUDITORY NERVE. gg 

III. Meniere's Disease — Meniere's Vertigo — Vertigo ab 

AURE L.ESA — VERTIGO IN GENERAL. 

When we speak of Meniere's disease we mean a combina- 
tion of symptoms which is made up (i) of subjective noises in 
the ear, (2) a feeling- of dizziness, accompanied with vomiting, 
(3) a gradually increasing difficulty of hearing, sometimes end- 
ing in deafness. 

On account of the exceptionally practical importance which 
has to be attributed to the so-called vertigo {le vcrtige, ScJiwin- 
delgefiihl), we may be allowed to make some general remarks on 
this before considering the special form, viz., Meniere's disease. 

By vertigo we mean a subjective feeling of motion appearing 
suddenly or gradually without any loss of consciousness, at- 
tended by a simultaneous sensation of loss of equilibrium. The 
subjective sense of motion is either referred to the body or parts 
of it, or to surrounding objects. The motion is in different 
directions, sometimes in horizontal or vertical circles, revolving 
with their convexity sometimes forward, sometimes backward, 
and the older observers distinguish accordingly a vertigo titu- 
bans, fluctuans, etc., from the nutatio — that is, subjective move- 
ments in a straight line. As concomitant symptoms we note 
headache, especially in the back of the head, anxiety, tremor, 
cold sweat on the face, nausea, vomiting ; in grave cases, 
transient clouding of consciousness, as in the prodromal state of 
an apoplectic attack. If consciousness is completely retained, 
as happens in the majority of cases, the subjective sensation of 
movement often gives rise to objective voluntary movements, 
to be regarded in a measure as instinctive efforts against the 
threatening danger of falling. The patient plants his feet firm- 
ly on the ground, stretches out his arms into the air, seizes 
with his hand any object within his reach, etc., but, in spite of 
all, he may, notwithstanding the perfect retention of conscious- 
ness, fall, owing to the feeling of disturbed equilibrium — ver- 
tigo caduca. 

If the patient is unconscious — e. g., asleep — then he experi- 
ences a sensation of falling down from a great height, down 
steps, or out of the window ; he imagines himself sinking into 
an opening in the ground, etc. This so-called nocturnal ver- 
tigo (Traumschwindel) usually torments those who suffer from 
vertigo when awake. Two exquisite examples of this vertigo 
I have observed in Bright's disease. 



IOO DISEASES OF THE CRANIAL NERVES. 

By far most commonly the vertigo occurs in paroxysms 
which appear without regularity and are of variable duration. 
Between the first and second sometimes hours and days, more 
rarely months, and indeed whole years, intervene, and only 
exceptionally — e. g., in cerebellar affections — do the subjective 
sensations of movement persist uninterruptedly, and thus render 
the vertigo constant. 

The position of the body has rarely any influence on the 
vertigo, for although at times some amelioration is felt on sit- 
ting down, there are cases in which the vertigo continues even 
when the patient occupies the horizontal position in bed. The 
pathogenesis of the trouble — that is to say, the organic changes 
in the brain which are necessary for the production of the sen- 
sation — are but little understood. It is generally supposed 
that changes in the blood-pressure, due, perhaps, to stimulation 
or paralysis of the vaso-motor nerves, are the chief cause of 
vertigo, just as a lasting decrease or increase in the amount of 
blood in the brain can probably give rise to attacks of dizziness. 
Until the conditions under which vertigo can appear in other- 
wise healthy people are more accurately understood, our 
knowledge of the pathological influences at work can be only 
imperfect. Of great interest are the experiments of Purkinje, 
undertaken seventy years ago, as to the influence of swing- 
ing, and especially of circular movements, in the production of 
vertigo. These were published in Rust's Magazin fur die ge- 
sammte Heilkunde, part xxiii, 1827, and have been reprinted in 
Romberg's Nervenkrankheiten (Joe. eit., p. 118) with this addi- 
tion by the author: "From all these experiments we see that, 
taking the head as a sphere, around the axis of which the true 
motion takes place, an imaginary plane through it determines 
in every case the apparent motion of the objects in the subse- 
quent position of the head at rest. The same holds good in 
attacks of vertigo." 

Johannes Muller also has made experiments on vertigo, and 
is inclined to attribute it to the after-effects of visual impres- 
sions on the retina. That this, however, is not always the case 
is shown by the fact that vertigo may appear in people whose 
eyes are closed, and even in the blind. 

We have already spoken about its occurrence in the paral- 
ysis of the ocular muscles (p. 48). Here let us add that this 
ocular or visual vertigo disappears if the patient closes the 
affected eye or holds his head in such a position that the 



DISEASES OF THE AUDITORY NERVE. IOI 

paralyzed muscle does not come into play during the act of 
seeing. 

In the present chapter we shall discuss more especially how 
far diseases of the internal and middle ear are connected with 
vertigo. It has repeatedly been observed that affections of the 
nasal mucous membrane, swellings of the erectile tissue, as well 
as affections of the mucous membrane of the larynx associated 
with violent cough (laryngeal vertigo), have produced it. If, 
then, we add that it has been claimed that the intestines (in- 
testinal worms, taenia, ascaris) and the stomach are responsible 
for feelings of dizziness, which Trousseau calls "vertigo a 
stomacho Iceso" we can not fail to be impressed with the com- 
plexity and the lack of clearness in the aetiology of this affec- 
tion. We must, however, always keep in mind, no matter where 
the remote cause lies, be it in the faulty movements of the 
ocular muscles, in the nose, in the ears, or in the stomach, etc., 
we must keep in mind, I say, that the influence of the cerebrum 
and the cerebellum is under all circumstances quite essential 
for the production of vertigo. Whether the characteristics cf 
the vertigo vary or not with the different organs affected is 
not yet clearly known. 

The disease described by Gerlier in Ferney, which shows itself by 
a very pronounced dizzy feeling, appearing in paroxysms — the so- 
called " paralyzing vertigo" — is accompanied by other symptoms, 
namely, a weakness, resembling a paralysis, in the extremities, droop- 
ing of the eyelids, and extraordinary lassitude without any loss of 
consciousness. This condition, which has been repeatedly observed 
in the canton of Geneva, where it occurs epidemically among labor- 
ers and herdsmen, is serologically mysterious. Gerlier attributes it 
to miasmata from marshes and stables, Eperon to the working in the 
sun which produces hyperemia of the meninges (Revue, med. de la 
Suisse romande, 1889, ix, 1) ; but neither of these hypotheses explains 
the immunity of the female sex. For this new and as yet entirely 
strange neurosis Gerlier has proposed the name vertige paralysant 
(Progres med., 1887, 26; Ladame, Revue med. de la Suisse romande, 
January, 1887; Deutsche Med. Zeitung, 1887, 44, 1888, 24). 

Middle life and moderately advanced age (especially in the 
female sex, and so in them the climacteric period) seem to pre- 
dispose to attacks of vertigo, which chooses by preference its 
victims from among vigorous and full-blooded individuals. Its 
frequent occurrence in advanced old age will not surprise us if 



I02 DISEASES OF THE CRANIAL NERVES. 

we remember the atheromatous condition of the arterial walls 
and the consequent irregularities of the blood supply to the 
brain substance. Among the exciting causes, poisons — e. g., 
tobacco (smokers' vertigo) — unaccustomed circular rocking 
movements, such as we feel on board ship, play an important 
role ; yet it is by no means clear how these causes act, and 
every attempt to explain, for instance, the nature of sea-sick- 
ness, or to prevent and cure it, has thus far been futile (cf. 
Pampoukis, Etude pathogenique et experimentale sur le vertige 
marin, Arch, de Neurol., 1888, xv, xvi). The dizziness ex- 
perienced on looking down from a height — the " height dizzi- 
ness " — which has erroneously been attributed to a fear of 
danger, is probably a reflex movement evoked by a wrong 
conception of our position in space, the result of a purely 
optical illusion ; for its production not only the cerebrum and 
cerebellum, but also the action of the retina, is needed. 

The prognosis in vertigo depends upon the nature of the 
primary disease, and Boerhaave's expression, " vertigo est om- 
nium morborum capitis levissimus et facillime curabilis," has 
to be taken cum grano salts. In an organic lesion of the cere- 
bellum — or more especially of the vermis — we can expect no 
improvement in the vertigo, while if it is attributable to an 
anasmia of the brain, occurring as a symptom of a general 
anaemia, the outlook is decidedly favorable. 

In the same way the treatment will be different in different 
cases according to the primary disease, which always has to 
be taken into consideration. For the symptomatic or prophy- 
lactic treatment, the repeated administration of mild laxatives, 
the frequent use of strong stimuli to the skin, such as cold 
douches, brushing of the neck and the back, mustard plasters, 
regular bodily exercise, and well-regulated diet, are to be rec- 
ommended, while any overloading of the stomach, especially 
in the evening, should be strenuously avoided. In spite of the 
much-lauded remedies (cocaine, etc.), we do not possess any 
reliable medicinal treatment for sea-sickness and height diz- 
ziness. 

After this digression we will return to the consideration of 
that form of vertigo which is especially connected with aural 
disturbances. Notwithstanding the fact that it is by no means 
settled that the above-mentioned combination of symptoms 
constituting Meniere's disease can be produced by a pure neu- 



DISEASES OF THE AUDITORY NERVE. yo ^ 

rosis of the auditory nerve, we will take it up here, because 
under all circumstances this nerve plays a prominent part in 
the pathology of the affection. 

Since Meniere in 1861 first described the disease, it has been 
repeatedly observed and carefully studied by German physi- 
cians. All have, however, failed as yet to give us a clear un- 
derstanding of its pathology. Meniere himself believed that 
an extravasation of blood or an acute exudation takes place 
into the labyrinth, which produces the same symptoms as 
occur in animals after injury to the semicircular canals. This 
view is in so far incorrect in that cerebral affections, accumula- 
tions of cerumen, and diseases of the middle ear, can undoubt- 
edly produce the same symptoms ; and then we have to re- 
member that not the haemorrhage nor the exudation, but its 
action upon certain parts of the membranous labyrinth is ne- 
cessary before the svmptoms occur (Politzer). It can easily be 
imagined that, whenever the extravasation stimulates the nerves 
of the ampullae, Meniere's symptoms are produced, while they 
are absent if the haemorrhage does not directly press upon the 
nerves of the antrum or the ampullae (Politzer). 

More recently Brunner (cf. lit.) has put forward a supposi- 
tion which we think is w r orth considering, namely, that we mav 
be dealing with a vaso-motor neurosis of the vessels of the laby- 
rinth. According to him, the pressure in the labyrinth acts 
in a similar way as pressure in the cranial cavity, where con- 
siderable changes are borne so long as the normal expansion 
of the subdural and subarachnoid space is not interfered with. 
He thinks, therefore, that narrowings of the labyrinth could 
produce a predisposition to Meniere's disease. 

This hypothesis is extremely plausible, especially as the 
symptoms appear paroxysmally, and in the intervals the patient 
is apparently in perfect health. In this way also the favorable 
action of quinine can be explained if we suppose that it dimin- 
ishes the hyperaemia in the semicircular canals, just as Horner 
has shown to be the case for the retinal vessels. He observed 
that large doses of quinine constantly produced considerable 
ischaemia in the latter. The question is, however, by no means 
settled, especially since cases have been observed where, in 
spite of the absence of the circular canals (Politzer), or in spite 
of the fact that they were filled with blood-clots (Lucae), no 
disturbances of equilibrium were noted during life. Hence it 
may also be possible that vertigo can be produced by pressure 



io4 



DISEASES OF THE CRANIAL NERVES. 



changes within the cranial cavity (Steiner, Deutsche med. 
Wochenschr., 1889,47). 

The view expressed by Peugnier and Fournier (cf. lit.) that 
Meniere's vertigo is a cerebral affection, and is only met with 
in individuals who are already insane or who will later surely 
become insane, certainly needs further confirmation and does 
not at all agree with our experience. 

There are hardly two cases in which the symptoms are ex- 
actly the same, and the course is so far from being uniform 
that we can not be surprised if often great uncertainty about 
the diagnosis prevails. The onset even is very variable. Now 
it is sudden, with loss of consciousness and apoplectiform symp- 
toms, etc. ; again it is gradual, first, subjective noises in the 
ears being noticed, sometimes comparable to the whistle of a 
locomotive, sometimes to the rustling of the leaves in the forest. 
Next comes a feeling of dizziness, at times only moderate, at 
times so pronounced that the patient in spite of all his efforts 
falls to the ground. Vomiting may be present or absent. 
Finally, a decrease in the power of hearing, first in one, then 
in the other ear, becomes noticeable. Some cases show a de- 
cided progressive tendency. After short remissions the symp- 
toms always reappear with increased severity, the vertigo 
gains so much in intensity that now the patient repeatedly falls 
with great violence, vomiting becomes more and more fre- 
quent, and the patient becomes at first incapable of following 
his calling, and finally is reduced to the state of a useless mem- 
ber of society. In rare instances periods of marked improve- 
ment, which may indeed last for years, occur. In these even 
the difficulty in hearing may be gradually diminished, and the 
prognosis becomes relatively favorable. Finally, it is at times 
observed that with the full development of the deafness all the 
other symptoms, buzzing in the ears, vertigo, and vomiting, 
disappear. In other words, we have what we call a relative 
recovery or recovery with defect. In any given case we are 
never in a position to predict the outcome, and have always to 
be very guarded in our prognosis. 

Of considerable diagnostic importance is the fact that usu- 
ally the examination of the drum and the Eustachian tube does 
not reveal any changes, and that neither cranial nor spinal 
nerves present any disturbances of function. Rinne's test 
gives variable results in Meniere's disease. This test consists, 
as is well known, in applying a vibrating tuning-fork with mod- 



DISEASES OF THE AUDITORY NERVE. 



105 



erate pressure first over the mastoid process, leaving it there 
until the patient no longer hears the sound, and then as quickly 
as possible bringing it immediately in front of the external 
meatus, avoiding all contact with the head or ear. If the pa- 
tient then is able to hear the sound of the tuning-fork once 
more, this is a sign that, as is normally the case, the conduction 
through the air is better than through the bone. If, on the 
other hand, he does not hear it, the conduction through the air 
must in some way be interfered with. In the diagnosis these 
are points to be considered. 

In the treatment, above all, the action of large doses of 
quinine — 0.7-1.0 (gr. x-xv) pro die — must be tried, a procedure 
warmly recommended by Charcot, and later used with gratify- 
ing results by Fere, Moos, and others. In many cases, as we 
have said, the effect is very marked, and there is no need to 
seek further for other medication. At times, however, this will 
fail, and then we are forced to resort to a two-per-cent solution 
of pilocarpine (nine to ten drops subcutaneously). The result 
is often surprising. I have seen grave symptoms completely 
subside after three or four days' use of this medicine. The in- 
jections are to be continued every second day for three or four 
weeks, and, as a rule, after the fifteenth dose the treatment can 
be discontinued, at any rate for a time. We need not add that 
on administering this drug the general condition of the patient 
must be carefully looked after, and any symptoms of collapse 
guarded against by the timely exhibition of stimulants, wine, 
and the like. Whether the view of Field (British Med. Jour- 
nal, 1890, xvii, 5) that the action of pilocarpine is to be attrib- 
uted to an increased secretion of cerumen is correct or not is as 
yet uncertain, although it must be acknowledged that in all 
cases of labyrinthian deafness the cerumen is absent. 

LITERATURE. 

Moos. Ueber Meningitis cerebrospinalis epidemica, insbesonclere iiber die nach 
derselben zuriickbleibenden combinirten Gehors- u. Gleichgewichtsstorun- 
gen. Heidelberg, 1881. 

Moos. Erkrankungen des Gehororganes bei Locomotivfuhrern und Heizern. 
Zeitschr. f. Ohrenheilk., x, 4, 1881 ; xi, 2, 1882. 

Gottstein u. Kayser. Breslauer arztl. Zeitschr., iii, 18, 1881. 

Burkner. Arch. f. Ohrenheilk., xvii, 1, 2, p. 8, 1881. 

Jacoby. Ibid., xvii, 4, p. 258, 1881. 

Burckhardt-Merian. Schweizer Correspondenzbl., xiv, I, 6, 1884. 

Roosa. Zeitschr. f. Ohrenheilk., xiii, 2, 3, 1884. 



106 DISEASES OF THE CRANIAL NERVES. 

Finkelstein. Wratsch, No. I, 1886. 

Baginsky. Ueber Ohrerkrankungen bei Railway-spine. Berliner klin. Wo- 

chenschr., 3, 1888. 
Loeb. Ueber clen Antheil des Hornerven an clen nach Gehirnverletzungen 

auftretenden Zwangsbewegungen, Zvv'angslagen und associerten Stellungs- 

anderungen der Bulbi und Extremitaten. Pfluger's Arch., 1892, 1, p. 66. 
Kreidl. Beitrage zur Physiologie des Ohrlabyrinths auf Grund von Versuchen 

an Taubstummen. Ibid., 1892, li, p. 119. 

Meniere's Disease. 

Charcot. Klinische Vortrage iiber Krankheiten des Nervensystems. Deutsch 

von Fetzer, Abthlg. ii, p. 343. Stuttgart, 1878. 
Guye. Arch. f. Ohrenheilk., xvi, 1, 2, 1880. 
Fere et Damars. Revue de med., i, 10, 1881. 

Woakes, Edward. Remarks on Vertigo and the Group of Symptoms some- 
times called " Meniere's Disease.'' Brit. Med. Journ., April 28, 1883, p. 

801. 
Bechterew. Neurol. Centralbl., 9, 1887 (anatomical study on the origin of the 

auditory nerve). 
Brunner. Zum Morbus Meniere. Zeitschr. f. Ohrenheilk., xvii, 1, 2, p. 47, 

1887. 
Grasset. Du vertige cardio-vasculaire ou vertige des arterio-scleraux. Clin. 

m£d. de l'Hop. Saint-Eloi, Paris, 1890. 
Buzzard. Lancet, 1890, i, 4, p. 179. 
Peugnier et Fournier. Vertige de Meniere, etc. Revue de med., 1890, 11; 

1891, 2, 3, 4. 
M tiller. Ueber Labyrinthschwindel. Deutsche Med. Ztg., 1893, No. 1, p. 9. 



CHAPTER VII. 

DISEASES OF THE GLOSSO-PHARYNGEAL NERVE. 

The glossopharyngeal nerve leaves the brain between the root 
fibres of the auditory and those of the vagus, at the side of the 
medulla oblongata, by five or six filaments ; these soon unite to form 
an anterior (small) and a posterior (larger) bundle ; they both pass 
outward, under and in front of the flocculus, to the anterior division 
of the jugular foramen, through which the nerve leaves the skull. 
Whether the so-called jugular ganglion which the nerve presents 
while still inside the skull has to be looked upon as a special gan- 
glion or only as a group of nerve-cells which have separated them- 
selves from the petrous ganglion, which is seen on the nerve immedi- 
ately after its exit from the skull, remains to be decided. 

The glosso-pharyngeal has no nucleus of its own, but originates 
in a large collection of nerve cells, which are regarded as the nucleus 
common to this nerve, the vagus, and the accessorius. This nucleus 
is situated midway between the anterior and posterior spinal roots. 
In the manner in which its root fibres originate it corresponds partly 
to the motor, partly to the sensory type (Wernicke). It is therefore 
designated as the mixed lateral system (Deiters), and it is supposed 
that the glosso-pharyngeal originates in the upper, the vagus in the 
middle, and the accessory in the inferior portion of the nucleus (cf. 
Fig. 17, p. 96). The manner in which this common nucleus is com- 
posed is not yet understood, nor do we know how many modes of 
origin for root fibres of this "lateral mixed system" we have to as- 
sume. Exact data may be found in Wernicke's text-book, i, p. 155 
ei seq. 

The glosso-pharyngeal, which, according to our present 

ideas, has to be regarded as the only genuine nerve of taste, is 

the third one which is to be taken into consideration in the 

examination of the functions of taste. The trigeminus (the 

third branch (lingual), possibly also the second branch) and the 

facial (chorda tympani) we have treated of, and it remains, 

therefore, to determine whether and if so under what condi- 

107 



IQ 8 DISEASES OF THE CRANIAL NERVES. 

tions diseases confined to the glossopharyngeal occur, and in 
what manner taste is altered by them. Since it only supplies 
the posterior third of the tongue with sensory fibres (ramus 
lingualis nervi glosso-pharyngei), it is not to be wondered at 
that, in determining an isolated affection of the nerve, we not 
rarely meet with considerable difficulties. 

We know but little about central diseases of this nerve. It 
is supposed, however, that there exists a bulbar affection, a 
gray degeneration of the nucleus which is found in tabes (Er- 
ben), also that the gustatory paths may be in a state of irrita- 
tion which gives rise to alterations in taste-perception analogous 
to the paresthesias which occur with irritation of the paths of 
tactile sense in the posterior columns of the cord. Conduction 
anaesthesias are also said to occur, although it is impossible to 
decide whether only the glosso-pharyngeal or whether in ad- 
dition the trigeminal and the facial paths are concerned (cf. 
Frankel, Berl. klin. Wochenschr., No. 3, 1875). A central 
paralysis of taste manifesting itself solely on the posterior third 
of the tongue has never been observed. With the cortical 
centre of the glosso-pharyngeal we are not as yet acquainted. 

Peripheral anaesthesia, anaesthesia gustatoria, ageusia (a 
priv., yevcns, sense of taste), impairment or loss of taste pro- 
duced by affections of the peripheral nerve endings, has been 
met with in diseases of the mucous membrane of the tongue, 
and has been known to be produced by the action of low tem- 
peratures (ice) or acrid substances (vinegar, chewing tobacco, 
red pepper). In testing for such alterations the patient is asked 
to close his eyes, open his mouth widely, and protrude his 
tongue ; then a small portion of sugar or quinine, etc., is placed 
upon that part of the tongue the function of which is to be 
tested, and the patient is to indicate with his finger where he 
perceives the taste before he retracts his tongue, and tell us by 
signs what he has tasted. The test is made with bitter, sour, 
sweet, and salty substances, and for the purpose any one, as 
long as it is not poisonous, may be selected. Further, it is pos- 
sible to accurately determine the boundaries of the area with 
normal and that with disturbed function of the tongue by means 
of the galvanic current. As Ave know, a sour, metallic, the 
so-called galvanic taste is perceived if the electrode is placed 
upon the tongue and the current is closed; the same taste is 
experienced during galvanization of the throat, the neck, or 
head, and is probably produced by the current acting upon the 



DISEASES OF THE GLOSSO-PHARYNGEAL NERVE. iQ g 

taste nerves in their peripheral or central course. The use of 
the galvanic current is also to be recommended in the treat- 
ment of the affections of the nerve. The spontaneous appear- 
ance of a sweet or sour taste in the mouth (parageusia) has 
often been observed in cases of diabetes, though we are igno- 
rant of the cause of this symptom. Therapeutically the leaves 
of Gymnema sylvestre, or the gymnemic acid contained in them, 
have been recommended in this condition (1$ Acidi gymne- 
mici (Merck), o.i (1.5 gr.) ; spir. vin., 9.5 ( 3 ijss.) ; theas nigr. 
Pekoe, 4.0 (3j); exsicca leni calore ; scatula lignea. D. S. : 
One to two wafers to be taken into the mouth repeatedly dur- 
ing the day and allowed to melt (Oefele, Aerztl. Rundschau, 
1893, Nos. 37, 38). 

LITERATURE. 

Romberg. Op. cit., pp. 148 et seq. 

Erb. Handbuch der Krankheiten des Nervensystems, pp. 219 et seq., 1876. 

Heusner. Eine Beobachtung iiber den Verlauf der Geschmacksnerven. Ber- 
liner klin. Wochenschr., No. 44, 1886. 

Zenner. Klinischer Beitrag iiber den Verlauf der Geschmacksnerven. Neurol. 
Centralbl., 1888, Nr. 16. 

Pope. Thrombosis of vertebral artery pressing on glosso-pharyngeal nerve; 
unilateral loss of taste at back of tongue. British Med. Journ., November 
28, 1889. 



CHAPTER VIII. 

DISEASES OF THE VAGUS (PNEUMOG ASTRIC NERVE), " VAGUS 

NEUROSES." 

Immediately behind the superficial origin of the glossopharyn- 
geal on the postero-lateral aspect of the medulla oblongata, the vagus 
appears, with its ten to fifteen separate bundles, which soon unite to 
form one trunk. This is a flat band which, accompanied by a process 
of the dura, passes outward below the flocculus, together with the 
accessorius, to the anterior division of the jugular foramen, inside of 
which is to be found the ganglion of the root of the vagus, or, as it 
is also called, the jugular ganglion. After its exit from the skull 
the vagus receives a part of the accessorius, and forms the gangli- 
form plexus or the ganglion of the trunk, which, however, only trans- 
mits a part of its fibres. 

About the difference in the further course of the left and right 
vagus we shall have a few words to say later. 

That the nucleus of the vagus is only a part of the nucleus com- 
mon to it, the glossopharyngeal, and the accessorius, has already 
been stated in the preceding chapter. The cells of the part belonging 
properly to the vagus are spindle-shaped, multipolar, 30 to 45 p long 
and 12 to 15/x broad (hence much smaller than the cells of the hypo- 
glossus nucleus, which we shall describe later). As another impor- 
tant origin of the root fibres of the vagus, a compact round nerve 
bundle following the longitudinal axis of the medulla oblongata must 
be mentioned. It has been described by Meynert as the solitary fas- 
ciculus, while Krause designates it as the respiratory fasciculus, be- 
cause it connects the vagus with the origin of the most important 
respiratory muscles (cf. Fig. 18). The so-called nucleus ambiguus (in 
the diagram n. am) is held to be still another nucleus of the vagus. 
This is a collection of peculiar nerve cells situated within the forma- 
tio reticularis to the mesial side of the nucleus lateralis. 

Just as most of the cranial nerves, the vagus may be dis- 
eased in its centre as well as in its peripheral course. The 
first class of cases are usually met with as partial manifesta- 



DISEASES OF THE VAGUS (PNEUMOGASTRIC NERVE), in 



tions of other, general, diseases (tabes, hysteria). The latter 
are distinct affections in themselves, which may occasionally 
be due to peripheral causes, such as indigestion, catching cold, 
or reflex influences, diseases of the intestines and the uterus. 
Ver)^ frequently, it is true, the seat of the disease remains ante 
as well as post mortem obscure, and this is not to be won- 
dered at if we remember that 
we know little or nothing 



n.X \ J? 

Ti.xir 




about the pathological anato- 
my of the vagus. Among the 
cases hitherto observed, many 
were not fitted to throw any 
light on the symptoms mani- 
fested during life, as in nu- 
merous instances no abnor- 
mality at all was found in the 
nerve, so that we are led to 
assume that the disease was 
purely functional (i. e., a dis- 
ease without appreciable ana- 
tomical basis). The pathol- 
ogy of the vagus, therefore, 
belongs to the most obscure 
chapters in the pathology of 
the cranial nerves, and the 
following can only be consid- 
ered to be an imperfect at- 
tempt at giving a compre- 
hensive exposition of the high- 
ly interesting diseases con- 
nected with this nerve. 

Since the symptoms may sometimes be the same whether 
the disease is of central or peripheral origin, we shall, so as to 
avoid repetition, deviate from our usual method of division, and 
give our attention chiefly to the question how lesions of the 
vagus may influence (a) respiration, (b) circulation, (c) digestion, 
functions which, as is well known, are chiefly under the con- 
trol of this nerve. 



/;*** n.ar. 

Fig. 18.— Cross-section through the Me- 
dulla Oblongata. (After Schwalbe.) 
a. V, ascending- root of the fifth. n.XII, 
nucleus of the hypoglossus. n.X and n.X', 
nucleus of the vagus. XII, hypoglossal 
nerve, fs, solitary funiculus (respiratory 
fasciculus), p, pyramidal tract, o, olive. 
z'. X, pyramidal nucleus, f.l.a., anterior lon- 
gitudinal fissure, n. am, nucleus ambiguus. 
n. /., nucleus lateralis. 



112 DISEASES OF THE CRANIAL NERVES. 

A. Affections of the Air-passages due to Lesions of 

the Vagus. 

i. The larynx, above all, interests us in this connection. This 
organ is innervated by the vagus and the accessorius, though it is 
still a matter of doubt whether all the motor fibres originate from 
the latter or only those that innervate the muscles used in the pro- 
duction of voice, while the vagus presides over the respiratory move- 
ments of the vocal cords ; the sensory fibres of the larynx certainly 
all belong to the vagus. 

The branches of the vagus, which come off in the cervical portion 
of the nerve and innervate the laryngeal muscles, are the superior 
laryngeal and the inferior or recurrent laryngeal. The former leaves 
the vagus at the lower end of the gangliform plexus, and divides into 
a motor branch, which goes to the crico-thyroid muscle, and into a 
sensory branch, which contains the fibres for the mucous membrane 
of the epiglottis and the whole laryngeal mucous membrane above 
the vocal cords. 

The recurrent laryngeal is shorter on the right side, because, with- 
out going beyond the upper aperture of the thorax, it curls around 
the subclavian artery, and runs back in a groove between the trachea 
and the oesophagus upward to the larynx, while on the left side it has 
to make the long course around the arch of the aorta. Its terminal 
branch (R. terminalis) divides into two twigs, which together supply 
all the muscles of the larynx, with the exception of the above-men- 
tioned crico-thyroid, with motor nerves, and the mucous membrane of 
the parts below the vocal cords with sensory fibres. 

Of the laryngeal muscles, the posterior crico-arytenoids draw the 
vocal cords apart — that is, they are the abductors or openers — while 
the lateral crico-arytenoids in conjunction with the lateral thyro- 
arytenoids draw them together, and are therefore called adductors or 
closers. Of these muscles, on each side the " abductor " arises at the 
posterior surface of the cricoid cartilage and passes upward and out- 
ward to the end of the muscular process of the arytenoid cartilage, 
while the other, the " adductor," arises from the upper margin of 
the cricoid cartilage and is inserted at the outer side of the muscular 
process of the arytenoid cartilage. It moves the muscular process 
forward, being thus the antagonist of the abductor. The crico- 
thyroids provide for the elongation and tension of the vocal cords; 
they are assisted by the internal thyro-arytenoids, which run parallel 
with the vocal cords. 

In the laryngeal muscles paralysis and, though compara- 
tively rarely, spasms have been observed. 



DISEASES OF THE VAGUS {PNEUMOGASTRIC NERVE). 113 

The chief forms of paralysis, which we shall here consider, 
are (1) the paralysis of the recurrent laryngeal, in which case 
all the muscles supplied by this nerve are paralyzed (or weak- 
ened) ; (2) the so-called abductor paralysis — that is, paralysis of 
the posterior crico-arytenoids, the openers of the glottis ; (3) 
paralysis of the internal thyroarytenoids. 

Without going into the much-discussed and still unsettled 
question as to the mechanism of these paralyses, we have at- 
tempted to give a succinct and clear summary of the clinical 
symptoms, including the appearances found on laryngoscopical 
examination (cf. table on page 116). 

The existence of a cerebral centre for the laryngeal muscles 
is shown by the fact that in different cerebral affections — e. g,, 
pseudo-bulbar paralysis and certain brain tumors — but only in 
rare instances (Rouge, Progres med., 1892, 36), paresis or pa- 
ralysis of the vocal cords has been observed. In chorea adduc- 
tor paresis has been noted. A most curiously perverted action 
of the vocal cords has been observed by Krause in the course 
of hysteria; on inspiration they were approached, while on ex 
piration the glottis was wide and gaping. 

Another form of central paralysis is the nuclear. In com- 
plete paralysis of one vocal cord a lesion in the accessorius 
nucleus of the corresponding side has been found ; the usual 
cause of this, however, seems to be a peripheral affection of the 
trunk of the vagus, or of the recurrent laryngeal (by pressure, 
contusion, injuries, surgical operations, tumors, and aneurisms), 
yet we are not often in a position to speak with certainty as to 
the seat of the affection, and to say whether this is central or 
peripheral. The nature of the laryngeal paralyses which occur 
in general neuroses (hysteria, epilepsy), intoxications (lead), in- 
fectious diseases (diphtheria, dysentery, cholera), is quite ob- 
scure. The easiest to understand are those acquired through 
straining of the voice and diseases of the larynx itself (catarrh, 
perichondritis). (B. Frankel on mogiphonia, cf. lit.) 

The prognosis ought to be guided by the consideration of 
the nature of the primary affection, but we should also take into 
consideration the functions of the affected muscles, and not 
forget that, for instance, in abductor paralysis, danger of suffo- 
cation may arise at any moment. It is always unwise to pre- 
dict the exact time of recovery ; the course of such paralyses 
is usually very protracted. 

The treatment of most of the cases has to be conducted by 

8 



114 DISEASES OF THE CRANIAL NERVES. 

a specialist, and consists in touching the vocal cords with the 
sound (Rossbach), and in the external or intralaryngeal use 
of electricity. Faradization of the different laryngeal muscles 
necessitates a dexterity which can only be attained after a thor- 
ough acquaintance with the laryngoscopical technique. The 
general treatment of any primary affection need not be dis- 
cussed here. 

Spasms of the laryngeal muscles, we have said before, are 
very rare, and are in general, with the exception of the spasm 
of the glottis, of not much practical importance. Most fre- 
quently the spasm affects the adductors, and the condition then 
resembles very much that of abductor paralysis, with this excep- 
tion, that the spasm is generally quite transitory, while the paral- 
ysis is often of long duration. The aphonia spastica described 
by Schnitzler, a disturbance of co-ordination of the muscles of 
the vocal cords, which, on an attempt at phonation, contract 
spasmodically, is found occasionally in chorea and hysteria. 

The spasm of the adductors, which occurs especially in 
early childhood, is called spasm of the glottis (laryngismus 
stridulus, laryngospasmus, asthma thymicum sive Millari). Its 
paroxysms usually occur unexpectedly without external cause. 
They consist in the main in a total arrest of respiration lasting 
from several seconds to a minute and a half, and are ushered in 
by a deep inspiration which is accompanied by signs of suffo- 
cation. Only rarely does the child die during the attack ; 
usually a few deep, very audible respirations indicate the cessa- 
tion of the spasm, and the child seems completely well after a 
comparatively short while. No definite statement can be made 
with regard to the number and intensity of the individual at- 
tacks, because innumerable variations can occur. The anatom- 
ical seat of the disease is entirely unknown ; yet the fact that 
not rarely eclampsia or epilepsy complicates the affection rather 
speaks for the possibility of a temporary irritation of the corti- 
cal centre for the laryngeal muscles. The remarkably frequent 
occurrence of it in conjunction with rachitis has led to the idea 
(Elsasser) that we are dealing with a rachitic softening of the 
posterior part of the skull, which has rendered possible pressure 
upon the brain. Nothing definite is known about the cause. 
In the treatment early hardening of the child and rational 
nutrition play an important role. Robust, well - nourished 
children who can stand changes in temperature without at 
^>nce catching cold, etc., are hardly ever affected with laryngis- 



DISEASES OF THE VAGUS {PNEUMOGASTRIC NERVE). n - 

mus stridulus ; only delicate children with a convulsive tend- 
ency, who have been fed on farinaceous foods and other inap- 
propriate substitutes for the mother's milk, fall a prey to the 
disease. There is no medicinal treatment for the affection. 
During- the attacks we have to avoid the danger of suffocation 
by carefully watching the epiglottis, sprinkling the body with 
ice-water, brushing and tickling the soles of the feet. After 
the attack we may give nervines (belladonna, bromide) and, 
perhaps to avoid a too frequent repetition, narcotics (morphine, 
i to 3 milligrammes — gr. J / (x r 1 / 20 subcutaneously). The treatment 
of the rachitis should never be omitted. 

Sensory disturbances of the larynx manifest themselves 
either in anaesthesias, or, what is less common, in hyperaesthe- 
sias of the mucous membrane, and are especially found in the 
distribution of the superior laryngeal. Thev are not rarely 
combined with motor changes, paralysis or paresis of the pha- 
ryngeal muscles (cf. chapter xi), but often they appear alone. 
The most common form is the anaesthesia attending diphthe- 
ritic paralysis ; it is characterized by the absence of the re- 
flex gagging and cough which normally follow touching the 
laryngeal mucous membrane with the sound, the finger, or the 
laryngoscope. In such cases it may happen that the food on 
deglutition enters the larynx, and, through faulty closure of 
the glottis, can not be removed by coughing, and thus gives 
rise to dangerous attacks of choking, and even to aspiration 
pneumonia. The latter does not seem to occur in cases of 
purely hysterical anaesthesia. 

The hyperaesthesia is found in ulcerative processes, or in bad, 
acute catarrhs. Although it seems to play a prominent role in 
hysterical patients, it is in reality not present, but is erroneously 
said to exist by patients who are forever worrying themselves 
and finding new ailments, or is produced by autosuggestion. 

The anaesthesia calls for electrical treatment, galvanization 
of the larynx and the palatal muscles, the faradic brush to the 
throat, etc. To meet the hyperaesthesia, narcotic remedies may 
be of service, but in hysterical patients often no other treat- 
ment but a good sensible lecture is needed. 

LITERATURE. 

Wegener. Ueber Kehlkopfmuskellahmung als Symptom der Tabes. Inaug.- 

Dissert., Berlin, 1887. 
Frankel, Bernh. Ueber die Beschaftigungsschwache der Stimme, Mogiphonie. 

Deutsche med. Wochenschr., 1887. 



n6 



DISEASES OF THE CRANIAL NERVES. 



TABLE OF THE MOST COMMON PARALYSES OF THE LARYN- 
GEAL MUSCLES. 





Kind of 
paralysis. 


Occurrence. 


Symptoms. 


Ophthalmoscopic picture. 




Complete 


In compression 


Voice not clear. 


Vocal cords slightly ab- 




recurrent 


paralyses of the 


Patient is easily 


ducted, the so-called 




laryngeal 


vagus or the re- 


tired on talking. 


" cadaveric position " 




palsy. 


current laryngeal 


Coughing impos- 


(Fig. ig). In forcible 






(carcinoma oeso- 


sible. 


phonation the healthy 






phagi), often uni- 




cord reaches beyond 






lateral (left), as 




the middle line. Over- 






initial symptom of 




riding of the arytenoid 






aortic aneurism. 




cartilages (Figs. 20, 21). 






In tabes. 








Abductor 


In diseases of the 


If bilateral: extreme 


Glottis appears as a nar- 




paralysis 


nerve itself, the 


inspiratory dysp- 


row slit, becoming still 


< 


(paralysis of 


causes of which 


noea; if unilateral: 


narrower on inspira- 


o 


the posterior 


are often un- 


inspiration ham- 


tion (Fig. 22). In- 


> 


crico- 


known. 


pered,long-drawn, 


ability to abduct the 


Pi 

< 


arytenoids). 




noisy. Dyspnoea 


paralyzed vocal cord 








on the least ex- 


(Fig. 23). 






ertion. Speech 










but little affected. 




u 
erf 


Paralysis of 


In catarrhs of the 


Voice hoarse; speak- 


Glottis does not close 


the internal 


mucous mem- 


ing an effort. 


completely on phona- 


o 


thyro- 


brane of the lar- 




tion (Fig. 24). If at 


oi 
O 


arytenoids. 


ynx. After over- 




the same time the aryt- 


2 




exertion of the 




enoids are paralyzed, 


fe 
£ 




voice. In hys- 




the glottis presents an 


1— 1 




teria. 




hour-glass outline (Fig. 
25). Neither anterior 
nor posterior portion is 
closed, but the vocai 
processes are in their 
normal position. 




Adductor 


Rarely isolated. In 


Absolute absence of 


Nothing characteristic. 




paralysis 


hysteria. 


voice. Power of 






(paralysis of 




coughing retained. 






the lateral 




" Phonic paraly- 






crico- 




sis " (Turck). 






arytenoids). 








o w 


Paralysis of 


After diphtheria. 


Voice rough ; high 


Excavation of the vocal 




the crico- 




tones impossible. 


cords. Cords do not 


£ >; 

CO < 
1— 1 


thyroids. 




1 


vibrate visibly. 



DISEASES OE THE VAGUS {FNEUMOGASTRIC NERVE). 117 




Fig. 19.— Bilateral Paralysis of the 
Recurrent Laryngeal. l ' Cadaveric 
position " of the vocal cords. 




Fig. 21.— Paralysis of the Recur- 
rent Laryngeal on the Left Side 
(in inspiration). 




Fig. 20.— Recurrent Laryngeal Paraly- 
sis. Overriding of the arytenoid car- 
tilages. 




Fig. 22. — Paralysis of both Posterior 
Crico-arytenoids (in inspiration). 




Fig. 23.— Paralysis of the Right Post. 
Crico-arytenoid (in inspiration ). 




Fig. 24.— Paralysis of both Internal 
Thyro-arytenoids (acute laryngitis). 




Fig. 25. — Paralysis of both Internal Thyro-arytenoids, 

associated with paresis of the arytenoid muscle. 
Figs. 19-25. — Partly after Strumpell, partly after Eichhorst. 



Il8 DISEASES OF THE CRANIAL NERVES. 

Holmes, Gordon. Paralysis of the Abductors of the Vocal Bands. Lancet, Octo- 
ber 22, 1887. 

Kidd. Bilateral Paralysis of the Dilator Muscles of the Glottis, with Subsequent 

Paresis of the Constrictors. Lancet, July 16, 1887, p. 108. 
Kiissner. Zur Kenntniss der Vagussymptome bei der Tabes dorsal. Berliner 

klin. Wochenschr., No. 20, 1887. 
Ziemssen, v. Ueber diphtheritische Lahmung und deren Behandlung. Klin. 

Vortr., iv, Leipzig, Vogel, 1887. 
Newmann. Glasgow Med. Journ., September, 1887 (Vagus Symptoms in Cases 

of Aortic Aneurism). 
Eisenlohr. Zur Pathologie der centralen Kehlkopflahmungen. Arch. f. Psych. 

u. Nervenhk., 1888, xix, 2, 314. 
Schech. Ueber Recurrenslahmung. Miinchener med. Wochenschr., 1888, 

xxxv, 51. 
Landgraf. Charite-Annalen, 1888, xiii, p. 250 (Left-sided Paralysis of the 

Vocal Cords in Pericarditis). 
Heymann. Zwei Falle von Lahmung des M. crico-thyreoideus. Deutsches 

Arch. f. klin. Med., 1889, xliv, 6. 
Burger. Die Frage der Posticuslahmung. Volkmann's Samml. klin. Vortr., 

1892, Neue Folge, 57. 
Katzenstein. Ueber die Medianstellung des Stimmbandes bei Recurrenslah- 
mung. Virchow's Archiv, 1892, cxxviii, Heft i. 
Laehr, M. Deutsche med. Wochenschr., 1893, 45. 

2. The lungs receive from the thoracic portion of the vagus the 
pulmonary or bronchial nerves, the so-called anterior branches of 
which, in conjunction with filaments of the sympathetic, form a plexus 
on the anterior wall of the bronchus, and enter with the latter the 
lungs, while the posterior branches, together with those coming from 
the four upper thoracic ganglia of the sympathetic, are distributed 
in the same way on the posterior surface of the bronchus. They 
are the motor nerves for the unstriped muscles of the bronchial tree. 

The diseases of the bronchial nerves, which produce, as 
it seems, a faulty innervation of the circular muscles of the 
bronchi, give rise to the morbid condition which has lately 
been the subject of much controversy, and is described under 
the name 

Bronchial Asthma, Asthma Bronchiale s. Convulsivum s. Nervo- 
sum, Spasmus Bronchialis {Romberg). 

Pathology. — Opinions about the nature of bronchial asthma 
are still divided. While some (Stork, Frantzel) maintain that 
it is due to an acute swelling- of the bronchial mucous mem- 
brane, others (Bamberger, Wintrich) consider a tonic spasm of 
the diaphragm to be responsible for it ; still others (Trousseau, 
Biermer) believe it to be a vagus neurosis, supposing, in conse- 



DISEASES OF THE VAGUS {PXEUMOGASTRIC XERVE). ng 

quence of a disturbed innervation (vagus), a tonic spasm to take 
place in the circular muscles of the medium-sized and fine 
bronchi, thus producing an acute pulmonary emphysema. 
After Bert had shown, in 1870, by experiment that a contrac- 
tion of the medium-sized and finer bronchi could actually be 
produced by irritating the vagus, later Biermer worked out 
his theory so thoroughly, and has defended it so successfully, 
that, in spite of the objections recently raised by Schmidtborn 
(Volkmann's Samml. klin. Vortrage, 1889, No. 328), who con- 
siders a vascular spasm in the distribution of the pulmonary 
artery to be responsible, we are probably justified in accepting 
it as correct, especially as with its help all the characteristic 
symptoms, the sudden onset and the sudden disappearance of 
the attacks, the expiratory dyspnoea, the low position of the 
diaphragm, etc., can well be explained. It is clear that this 
bronchial spasm forms an impediment much more easily over- 
come by inspiration than by expiration, and that this difficulty 
in expiration must of necessity not only influence the alveoli, 
but also the smaller bronchi, from which the inspired air can 
only imperfectly be forced out ; hence arise dyspnoea and 
emphysema during expiration. On auscultation, sibilant rhon- 
chi are heard all over the chest. But all this does not explain 
the cause of the spasm. This may be sought for in an inde- 
pendent affection of the bronchial mucous membrane, a view 
which possibly may be supported by the presence in the sputa 
of asthmatics of the so-called " Curschmann's spirals " (spiral 
threads which must be looked upon as casts of the finest bron- 
chioles), and of so-called hsemosiderin cells found by v. Noor- 
den, which are identical with pigment cells (Zeitschr. f. klin. 
Med., xx, 1, 2). Or we may assume a reflex origin. Thus Ley- 
den maintained that certain pointed octahedral crystals which 
he discovered in the sputa of asthmatics irritated the mucous 
membrane, and produced the spasm. Many observations, how- 
ever, allow us to doubt the correctness of this latter view. 
It has been established, on the other hand, beyond doubt (Vol- 
tolini, Hack, Sommerbrodt), that certain diseases of the nasal 
mucous membrane (polypous growths, chronic catarrh, etc.) 
may give rise to asthmatic attacks — reflex neurosis ; possibly 
some part in the production of these is played by the reflex 
dilatation of the vessels in the bronchial mucous membrane, 
which was by Stork and Weber supposed to take place in con- 
nection with the bronchial spasm, a theory which was after- 



120 DISEASES OF THE CRANIAL NERVES. 

ward confirmed by Sommerbrodt. With reference to this con- 
nection I have convinced myself from long- experience with 
such cases that the above-mentioned affections only lead to 
asthma in persons with a nervous predisposition ; they are only 
the " agents provocateurs" not the real cause (Brissand, Revue 
de med., 1890, 12). This is especially the case in children 
(Blache, Etude sur l'asthme chez les enfants, Paris, 1890). 

Symptoms. — The characteristic features of the disease are 
the paroxysms of distress and dyspnoea, previous to which the 
patient may for days complain of general malaise, be low-spirit- 
ed, and troubled with digestive disturbances, diarrhoea, etc. 
The attacks begin quite suddenly, usually at night, more rarely 
in the daytime ; during them the respiration is changed, so that 
the breathing in inspiration, but more especially in expiration, 
becomes labored and accompanied by a loud wheezing. This 
may last only a few hours or may continue for days, and may 
be repeated at varying intervals. Toward the end of the attack 
moist rales can be heard on auscultation, and there is 4 expecto- 
ration which contains the above-mentioned spirals and crystals. 
Between the attacks the patient enjoys perfect comfort. 

etiology. — The aetiology of the disease is but little known. 
No doubt hereditary predisposition does exist, and persons 
with a neuropathic family history fall, cceteris paribus, more easy 
victims to asthma than others. Just of what nature the exciting 
causes of the actual outbreak are we are as yet unable to say. 
We have repeatedly observed that hysterical persons suffer 
from asthmatic conditions, which, on examination of the respir- 
atory organs, prove to be of a nervous origin. In these in- 
stances the patients are for days troubled with paroxysmal 
dyspnoea, their expiration is difficult and wheezing, while noth- 
ing abnormal is found on auscultation and percussion. We shall 
later on have more to say about this hysterical asthma. 

That the inhalation of certain kinds of dust may give rise to 
asthma, while not a frequent, is certainly a well-authenticated 
observation. We may especially find this connection when the 
same obnoxious causes have been acting frequently and through 
a rather prolonged period of time, as is the case in those who 
follow certain occupations (millers, bakers, etc.) ; in the same 
way it is well known that repeatedly druggists have been af- 
fected regularly with asthmatic attacks while occupied with the 
pulverization of ipecacuanha root, and that the dust of certain 
kinds of grain — for instance, of oats — causes such disturbances 



DISEASES OF THE VAGUS {PNEUMOGASTRIC NERVE). I2 i 

in those engaged in thrashing (cf. Hirt, Krankheiten der Ar- 
beiter, 1871, Bd. i, p. 12). 

The asthma which develops under the influence of certain 
poisons has to be classed among these cases, and in this con- 
nection the so-called lead asthma (asthma saturninum) is deserv- 
ing of special mention. This is a very peculiar disease, which 
sometimes sets in very acutely only a few minutes after the 
work has been taken up. Though to the highest degree dis- 
tressing to the patient, a fatal outcome in it has never been 
noted (cf. Hirt, op. cit., Bd. iii, p. 40). This trouble is, however, 
even among lead-workers, quite rare, so that we may assume 
that among one hundred affections due to working in lead two 
instances at most of this above-described asthma occur. As to 
the mode of origin, we do not know whether to refer it to the 
action of the poison on the central nervous system, or on the 
peripheral nerve-endings of the vagus. 

Treatment. — We are not acquainted with any specific for 
bronchial asthma; the much-recommended iodide of potassium 
(2.0-5.0 (xxx to lxxv grs.) a day) often fails, and, as a rule, we do 
not accomplish much with the usual nervines, arsenic, quinine, 
bromide, etc. From the use of electricity we have never seen 
any lasting benefit. Well-conducted hydrotherapeutic meas- 
ures may produce a decided decrease in the frequency and the 
severity of the attacks. For the treatment of the attack itself 
we can foremost recommend pyridin, which was suggested by 
See. It is a product obtained in the dry distillation of organic 
substances, a colorless fluid which easily evaporates in the air. 
For the therapeutic use half a teaspoonful of it has to be poured 
on a shallow dish, and this inhaled three to four times daily in 
a closed room. The smell is horrible, and often disgusting, but 
in many instances the action was found extremely beneficial. 
As soon as the pyridin evaporates the patient becomes easier, 
the feelings of distress are relieved, the heart's action is more 
regular. The effect is not always lasting ; still, I have seen 
cases in which daily regular inhalations used for several weeks 
have not only cut short the individual attacks, but have also 
decreased their frequency. Of course, with this, as with all 
other remedies, we may be disappointed. From the inhalation 
of the fumes of burning saltpetre paper, which has recently 
again been recommended by Kochs, I have only seen transient, 
never any lasting effects. The same holds for the well-known 
stramonium cigarettes, for amyl nitrite, and the vapors of tur- 



122 DISEASES OF THE CRANIAL NERVES. 

pentine. More good may be expected from the administration 
of tinct. lobelias, which often works like a charm (tinct, lobel., 
5.0 (tti lxxv) ; aquae laurocer., 15.0 (3iv). Sig. : 15 to 20 drops 
every two hours). An alkaloid " lobelin " has been used 
by Nunes (Rio de Janeiro, 1889). With the extract of que- 
bracho, which has been recommended by Penzoldt, I have no 
large experience of my own. Hyoscyamine, together with 
small doses of strychnine, given several times a day, has been 
used by Walker (Lancet, August 20, 1887, p. 368). 

LITERATURE. 

Biermer. Ueber Bronchialasthma. Volkmann'sche Sammlung klin. Vortrage, 

1870. 
Mahaux. The Pathogenesis of Asthmatic Attacks. Journ. de Brux., vol. lxxiii, 

p. 205, 1881. 
Riegel und Edinger. Deutsche Zeitschr. f. klin. Med., 1882. 
Hack. Ueber eine operative Radicalbehandlung bestimmter Formen von Mi- 

grane, Asthma, Heufieber, sowie zahlreicher verwandter Erscheinungen. 

1883. 
Schech. Die sogen. Reflexneurosen und ihre Beziehungen zu den Krankheiten 

der Nase und des Rachens. Bayer, arztl. Intelligenzbl., Bd. xxxi, p. 30, 

1884. 
Schaffer. Aus der Praxis : Nasenleiden und Reflexneurosen. Deutsch. med. 

Wochenschr., pp. 23, 24, 1884. 
Sommerbrodt. Mittheilungen von Heilungen patholog. Zustande, welche durch 

Reflexvorgange von der Nase her bemerkt wurden. Berl. klin. Wochen- 
schr., pp. 10, 11, 1884. 
Sommerbrodt. Ueber Nasenreflexneurosen. Ibid., No. 11, 1885. 
See, Germain. Bull, de Therapeut., June 30, 1885, vol. cviii, p. 529. (Recom- 
mends pyridin.) 
Biermer. Berliner klin. Wochenschr., 41, 1886. 
Kochs. Beitrag zur Kenntniss der Verbrennungsproducte des Salpeterpapieres 

und der Ursachen des Asthma bronchiale. Centralbl. f. klin. Med., Bd. vii, 

p. 40, 1886. 
Grocco, Pietro. Sulla patologia dei nervi cardiaci. Rivist. clin. di Bologna, p. 

12, 1886. 
Dusseaud. De l'asthme d'origine nasale. These de Paris, 1887. 
Calmettes. Les nevroses reflexes d'origine nasale. Progr. med., No. 28, p. 20, 

1887. 
Fraser. On Dyspnoea, especially on the Dyspnoea of Asthma and Bronchitis, 

and the Effects of the Nitrites upon it. Lancet, July 9, 1887, p. 51. 
See, Germain. Die einfachen Lungenkrankheiten. Translated into German by 

M. Salomon. Berlin, Dammler, 1887. 
Brugelmann. Ueber Asthma, Deutsche Medicinal-Zeitung, 29 et seq., 1888. 
V. Basch. Wiener Med. Ztg., 1888, 23, 24. 
Grancher-Barbier. De l'asthme chez les enfants. Gaz. med. de Paris, 

1880, 16. 



DISEASES OF THE VAGUS (PNEUMOGASTRIC NERVE). i 2 $ 

Bloch. Die sogenannte nasale Form desBronchialasthma. Volkmann's Samm- 

lung klin. Vortrage, 1889, No. 344. 
Schmidt, Ad. Zur Kenntniss des Asthma bronchiale. Centralbl. f. klin. Med., 

1 891, 25 (Examination of Asthmatic Sputa for Fibrin). 

B. Cardiac Affections due to Lesions of the Vagus. 

The superior and inferior cardiac branches are given off from the 
cervical and thoracic portions of the vagus ; they join the cardiac 
branches of the sympathetic to form the cardiac plexus (superficial 
and deep). It has not yet been determined of what character these 
fibres are; there is, however, no doubt but that we have to distin- 
guish inhibitory fibres, the stimulation of which diminishes, and 
accelerator fibres, the stimulation of which increases the number of 
heart beats. The sensory nerves of the heart are also furnished by 
the vagus. 

Angina Pectoris. 

Among the neuroses of the heart which probably are caused 
by a disturbance in the vagus, we shall first consider angina 
hysterica or angina pectoris (stenocardia, cardiac neuralgia, 
nervous heart pain), a disease of the true nature of which our 
knowledge is as yet quite imperfect, though its symptoms have 
been recognized for more than one hundred years (Heberden, 
1772). Its cardinal symptom is a piercing, burning, paroxysmal 
pain in the region of the left nipple, attended with a sensation 
of impending death ; it often radiates into the left arm, and even 
down to the finger tips, and may continue for minutes or hours. 
It usually begins without any premonition, and surprises the 
patient by day at his work, or wakes him up at night out of his 
sleep. The severity of the pain differs ; in some cases it is 
moderate, in others it reaches an insupportable degree. Dysp- 
noea is not always present ; the respiration remains sometimes 
regular and quiet, although the patient suffers from a distress- 
ing feeling of anxiety, and his skin is covered with a cold sweat. 
During the intervals, the patient feels perfectly well, unless 
there is a co-existing lesion of the heart muscle or valves. 

The diagnosis may present some difficulties, since interme- 
diate conditions between angina pectoris and bronchial asthma 
are met with, or a combination of the two conditions may occur. 

The prognosis depends mainly upon the question whether 
we have to deal with a vagus neurosis, or whether some com- 
plication co-exists. If the myocardium, owing to disturbed in- 
tracardial circulation (caused, for instance, by atheroma of the 
coronary arteries and insufficient blood supply to the myocar- 



124 



DISEASES OF THE CRANIAL NERVES. 



dium, or by syphilis), has undergone pathological changes, 
death may occur during an attack. Such cases are not rare, 
and I have recently again had occasion to observe an instance 
of this kind, in a man of robust appearance who suffered from 
stenocardia, and who, while in apparently good health, died 
suddenly in an attack within two minutes after its onset ; the 
arteriosclerosis was very pronounced. Sudden death, however, 
is never to be feared unless the heart is organically diseased. 
It is impossible to give an absolutely favorable prognosis with 
regard to recovery, because here also we do not possess any 
remedy which is capable of doing away with the attacks en- 
tirely. But the same suggestions as have been made for the 
treatment of bronchial asthma apply to cases of angina pectoris, 
and about the same results have been obtained in both. If in- 
ternal treatment can not be dispensed with, digitalis may in the 
first place be tried, then strophanthus, and finally arsenic, 
which latter may with advantage be combined with strychnine. 
With the tinct. piscidiae erythrinae, which is supposed to lower 
arterial tension and which has been recommended by Liegeois, 
I have no personal experience. It is prescribed as follows : 
Tinct. pise, erythr., 60.0 ( 3 xv) ; tinct. veratr. virid., 10.0 ( 3 ijss.) ; 
tinct. aconiti, 15.0 (ttl 225). Sig. : 15 to 20 gtt. t. i. d. 

For the attacks, freshly prepared amyl nitrite, a few drops 
(5 to 10), to be carefully inhaled by the patient, is the most use- 
ful treatment ; besides this, inhalations of chloroform and hypo- 
dermic injections of morphine deserve recommendation, as 
they relieve the patient at once from the intolerable torments 
of his condition. The severe states of collapse following these 
measures, observed by Bamberger, are probably, after all, quite 
exceptional. 

Murrell recommends a systematic treatment with nitro- 
glycerine (Therap. Monatshefte, 1890, iv, 11), beginning with 
0.000 1, increased gradually to 0.003 P- die. From external meas- 
ures, such as the application of hot- water bags or ice-bags over 
the heart, as well as from hot baths, I have seen no good result. 

The aetiology of the disease is as obscure as its nature : here 
we must again carefully discriminate between the cases where 
the angina pectoris is merely a symptom of some organic heart 
disease (disease of the coronary arteries, fatty heart, valvular 
disease), and where it appears as an independent affection — i. e., 
where no heart lesion can be demonstrated. The latter form 
is disproportionately less frequent (Gauthier). Males and those 



DISEASES OF THE VAGUS {PNEUMOGASTRIC NERVE). i 2 $ 

advanced in age seem especially predisposed to the disease 
(Gauthier) ; yet the author has also seen cases where displace- 
ment of the uterus was accompanied by stenocardia, as well as 
cases of undoubted angina pectoris in children thirteen to fif- 
teen years of age. Psychical disturbances, such as are found 
in hysterical patients, also the influence of certain poisons — 
e. g., tobacco — deserve some consideration. Peyer (Zurich) 
claims to have observed a connection between stenocardia 
and spermatorrhoea (Wiener med. Presse, 1892, 25). That an- 
gina pectoris is a vagus neurosis can reasonably be accepted, 
as the sensory fibres of the heart are furnished by the vagus, 
and as pain is the most prominent symptom of the trouble. 
Presumably the sympathetic is, however, also concerned, and 
some are even inclined with Lancereaux, who several times 
found this nerve vascularized, to regard the cardiac plexus of 
the sympathetic as the chief seat of the disease ; but even were 
this so, we could not exclude some participation of the vagus. 
A publication of Leroux, who found at the autopsy a bronchial 
gland and the right vagus grown together in a case where an- 
ginal seizures had existed until just before death, appears also 
to speak in favor of an implication of the latter nerve. Fre- 
quently no anatomical lesion can be found. 

LITERATURE. 

Lustig. Zur Lehre von den vasomotor. Neurosen (Angina pectoris). Inaug. 

Diss., Breslau, 1875. 
Balfour. Edinb. Med. Journ., March, 1881, vol. xxvi, p. 769. 
Mackenzie, John N. A Contribution to the Pathology and Treatment of the 

Respiratory Vasomotor Neuroses. New York Med. Journ., Feb. 26, 1887. 
Huchard. The Weekly Med. Rev., St. Louis, 7, 1887. (Recommends perse- 
verance with iodides — iodide of sodium, gr. xv-xlv daily for a year or a year 

and a half.) 
Le Clerc. L'angine de poitrine hysterique. O. Doin, Paris, 1887. 
Von Basch. Die cardiale Dyspnoe und das cardiale Asthma. Klin. Zeit- und 

Streitfragen, i, 3, 4, 1887. 
Gelineau. Traite de l'angine de poitrine. Paris, Delahaye, 1887. 
Honigmann. Beitrag zur Kenntniss der Herzneurosen. Deutsche med. Wo- 

chenschr., 1888, 45. 
Obelensky. Der Nerv. vagus und die Angina pectoris. Berliner klin. Wo- 

chenschr., 1889, 52. 
Frankel, Vierordt et al. Verhandlungen des X. Congresses fur innere Medicin. 

Deutsche Med.-Ztg., 1891, 39, p. 463 et seq. (Interesting discussion.) 
Frankel. Berliner klin. Wochenschr., 1891, 21. 
Lehr. Die nervose Herzschwache (Neurasthenia vasomotoria) und ihre Be- 

handlung. Wiesbaden, Bergmann, 1891. 



l 2 6 DISEASES OF THE CRANIAL NERVES. 

Nervous Palpitation of the Heart. 

Secondly, we have to speak of the so-called nervous palpi- 
tation of the heart, palpitatio s. hyperkinesis cordis, by which 
term we designate a paroxysmal increase in the frequency and 
strength of the heart's action, which is not only objectively 
noticeable, but also subjectively felt by the patient. Pain is 
absent, and in pure cases at least there is no dyspnoea. Palpi- 
tation appears more frequently as an independent affection than 
angina; the attacks usually begin suddenly, often at night. If 
the patient be lying on his left side, he is seized with a feeling 
of oppression and anxiety, the pulse is accelerated, and its rate 
may be increased to more than two hundred beats to the min- 
ute ; sometimes the second heart sound is curiously clicking 
(cliquetis metallique) and the first extraordinarily weak, the 
carotids throb, the radial pulse becomes hard and full. Dehio 
(cf. lit.) has examined the pulse curves by means of a Dudgeon 
sphygmograph, and found the pulse waves higher, the decline 
steeper, the first elastic elevation decidedly nearer to the base 
line of the curve, and the dicrotic elevation lower than normal. 
He attributes this condition to an increase in the frequency of 
the beats, and a decrease in the duration of the individual ven- 
tricular contraction. Besides the palpitation, the patient com- 
plains of ringing in the ears, dizziness, and faintness. The 
attacks usually pass off in a few minutes, disappearing as sud- 
denly as they came on, and the patient soon feels perfectly well. 
Their frequency is extremely variable ; they may appear once, 
twice, or more often daily, or only after long intervals of weeks 
or months. 

That here we also have to deal with a neurosis of the vagus 
seems only a rational assumption. The seat varies; it may be 
either central or peripheral, but in most cases we are unable to 
positively say which it is. Sometimes we are justified in as- 
suming that such conditions depend upon a central, bulbar 
nuclear affection, just as we may probably refer a temporary 
diminution of the vascular tonus to a transient paresis of the 
vaso-motor centre in the medulla oblongata (Dehio). The pub- 
lication of Cuffer (Revue de med., 1890, 4) shows that neuritic 
conditions of the vagus may also be found. 

It is very important in these cases of palpitation to look for 
further co-existing affections, after the removal of which the 
nervous palpitation often disappears suddenly, and never re- 



DISEASES OE THE VAGUS {PNEUMOGASTRIC NERVE). \ 2 J 

curs. To this class belong chiefly the anaemias of the young, 
cardiogmus juvenilis, habitual constipation, gout, and malaria, 
and accordingly we are able to bring about a marked improve- 
ment in the palpitation, which in such cases is only symp- 
tomatic, by improving the condition of the blood, by proper 
regulation of the bowels, by promotion of the excretion of uric 
acid, and by combating malaria by means of quinine, according 
to the indications in each. If such indications for therapeutic 
measures are wanting, we have to fall back upon the narcotics 
and nervines, unreliable as they are in their action. In hyster- 
ical persons certain mechanical manipulations, pressure on the 
abdomen, momentary compression on the neck, and the like, 
may be of service. iVpplication of the ice-bag to the cardiac 
region may occasionally be beneficial ; the psychical treatment 
of the patients, repeated assurances that these attacks are never 
fatal, and that they are quite amenable to treatment, is not to 
be underrated ; in the case of children especially this has been 
found very effectual. 

The aetiology is, unless the palpitation is secondary to an 
underlying disease, quite obscure. Under what conditions 
individuals in other respects quite sound, with a good family 
history, and who present no symptoms of neurasthenia, can 
be attacked by such transient pareses of the vagus we do not 
know. In suspicious cases we should think of masturbation. 

LITERATURE. 

Preisendorfer. Ueber reflectorische Vagusneurose. Deutsches Arch. f. klin. 
Med., xxvii, 3, 4, p. 387, 1880. 

Langer, L. Ueber Vaguslahmung. Wiener med. Wochenschr., xxxi, 30, 31, 1881. 

Pick. Prager med. Wochenschr., No. 44, 1884. 

Fothergill. The Gouty Neuroses of the Heart. Edinb. Med. Journ., xxx, p. 
393, November, 1884. 

Semmola, Mar. De l'ataxie paralytique du cceur, d'origine bulbaire. L'Ence- 
phale, vi, 6, p. 413, 1886. 

Dehio. Ueber nervoses Herzklopfen. Petersb. med. Wochenschr., August 2 
and 9, 1886. 

Mackenzie, Nol. A Contribution to the Pathology and Treatment of the Respira- 
tory Vaso-motor Neuroses. New York Med. Journ., February 26, 1887. 

Grodel. Ueber nervoses Herzklopfen und sonstige auf Innervationsstbrungen 
beruhende Herzaffectionen. Deutsche Med.-Ztg., 1890, 49. 

Tachycardia. 
In rare cases, in people otherwise healthy, but more fre- 
quently in those affected with heart disease, we meet with a 



I2 8 DISEASES OF THE CRANIAL NERVES. 

transient acceleration of the heart's action (tachycardia), which 
usually lasts for several hours, after which the pulse rate again 
becomes normal. These attacks are accompanied by a feeling 
of great anxiety, and are ushered in by vaso-motor disturbances 
— e. g., circumscribed flushings. The number of the pulse 
beats may reach 200 or more. Pressure upon the vagus in the 
neck, a draught of cold water, or similar stimulation of the 
peripheral ends of the vagus often may cut short an attack 
against which we possess no other remedy. Whether in a 
given case irritation of the accelerators or a paroxysmal paral- 
ysis of the vagus is responsible for the attacks has, according 
to Nothnagel (Wiener med. Blatter, i, 2, 3, 1887), to be decided 
in the following way : A great increase in the frequency of 
the pulse, accompanied by a weak heart-beat, and perchance 
another disturbance of some nerve path belonging to the vagus, 
speak for paralysis of this nerve ; whereas a strong impulse, 
fullness of the peripheral arteries, with high tension, associated 
with other symptoms of vaso-motor irritation, is in favor of 
stimulation of the accelerators. Traube assumes that some 
cases are due to a temporary anaemia in the medulla oblongata, 
in consequence of which a paresis of the inhibitory nerves en- 
sues. To this class seems to belong the case related by Dehio 
(cf. lit.). The affection is met with equal frequency in both 
sexes ; it is more liable to occur in advanced age ; in women 
the climacteric period seems to predispose to it (Stokes, Kisch). 

The mode in which nicotine acts upon the vagus is of 
great interest, and certainly deserves a closer study than has 
been given to it hitherto. 

Chronic nicotine poisoning, as it is found in smokers, and 
only occasionally in tobacco workers, is not always well adapted 
to throw much light on this subject, for, whereas it is well 
known that the nicotine when brought into direct contact with 
the nerves paralyzes them rapidly, it is by no means common to 
find paralysis of the vagus in the course of nicotine intoxication. 
As a rule, it is true that the heart's action is increased, yet cases 
occur in which there is a slowing, so that we are led to think of 
a stimulation of the vagus, such as happens after drinking cold 
water, where the pulse rate may be reduced to thirty or twenty 
beats. Owing to the miserable arrangements for ventilation in 
tobacco factories, we have from time to time occasion to study 
the action of nicotine in those employed in them, although the 
disease is, as has been said, by no means frequent. Kisch has 



DISEASES OF THE VAGUS {PNEUMOGASTRIC NERVE). i2 g 

recently called attention to a form of tachycardia which oc- 
curs at the menopause, and which he is inclined to attribute 
to changes in the ovaries (Wiener med. Presse, 1891, 19). 

Cases which, in consequence of a vagus neurosis, present a 
simultaneous disturbance in the circulatory and respiratory 
apparatus, occur, but are rather uncommon. A case to the 
point has been published by Tuczek (Deutsches Arch. f. klin. 
Med., 1877, xxi, 1), and two others by Kredel (ibid., 1882, xxx, 
p. 547). For the respiratory apparatus acute emphysema, with 
dyspnoea and symptoms of catarrh, were noted ; they were as- 
sociated with tachycardia (asthma cardiacum, according to 
Kredel), and the existence of a paralysis of the vagus fibres 
regulating the heart, in conjunction with a stimulation of those 
presiding over the lungs, whereby spasm of the muscles of the 
bronchi was produced, was assumed. At the autopsy the 
cause was found to be pressure exerted upon the vagus trunk 
by a rapidly swelling lymph gland. The attacks lasted from 
twelve to thirty-six hours. Some of the patients had organic 
heart disease. 

LITERATURE. 
Langer. Ueber Vaguslahmung. Wiener med. Wochenschr., xxxi, 30, 31, 188 1. 
Probsting. Ueber Tachycardie. Deutsch. Arch. f. klin. Med., Bd. xxxi, Heft. 3 u. 4. 
Dejerine. Neuritis of the Vagi consequent upon the Action of Alcohol. Soc. 

de Biol, de Paris, July 16 and 23, 1887. 
Dehio. Tachycardie nach der Punction eines Hydrops-Ascites. Petersb. med. 

Wochenschr., 2, May 14, 1887. 
Farvarges. Ueber die chronische Tabakvergiftung und ihren Einfluss auf das 

Herz und den Magen. Wiener med. Wochenschr., Nos. 11-14, 1887. 
Spengler. Deutsche med. Wochenschr., No. 38, 1887. 
Bristowe. Brain, xxxviii, p. 164, 1887. 
Brieger. Charite-Annalen, 1888, xiii, p. 193. 
Bouveret. Revue de med., 1889, 9, 10. 

Jouanneau. De la Tachycardie dans la Tuberculose pulmon. These de Paris, 1 889. 
Huchard. Revue gen. de clin. et de therap., 1890, 33. 
Sollier. Progres med., 1890, 37. 
Hurr. British Med. Journ., 1890, 15, 3. 

Bradycardia (fipaSvs, slow) is, on the whole, even less often 
met with than tachycardia ; in this condition the number of 
the pulse beats may fall to half the normal — i. e., to 38-42, a 
condition which may also be found in perfectly healthy indi- 
viduals. Sometimes bradycardia seems to be a peculiarity 
common to several members of the same family. After pro- 
longed fasting, and in the puerperal state, it may occur with- 
out any other abnormality. Sometimes it is associated with 
9 



130 DISEASES OF THE CRANIAL NERVES. 

cerebral affections, with chronic articular rheumatism, with dis- 
eases of the digestive, circulatory, and uropoetic organs, or with 
certain intoxications (lead, alcohol, coffee). Lunz, among others, 
has recently called attention to the association of bradycardia 
with epileptic attacks, the so-called Adams-Stokes disease (Neu- 
rol. Centralbl., 1893, xii, 4, p. 142). In old men it is sometimes 
seen as an idiopathic vagus neurosis, a condition for which no 
physiological explanation can be given (cf. Grob, Deutsch. 
Arch. f. klin. Med., 1888, xlii, p. 574; also Riegel, Zeitschr. f. 
klin. Med., 1890, xvii, 3, 4, p. 221 ; also Dehio, Petersburger med. 
Wochenschr., 1892, 1. In these articles also the theories of 
the pathogenesis of the affection are discussed). We have thus 
far no means with which to treat this condition successfully. 

It scarcely belongs within the scope of this book to treat of 
disorders of the cardiac rhythm, arrhythmia cordis, which is 
sometimes found in obesity, more often in the course of brain 
diseases, in intoxications (tobacco, coffee, digitalis), and above 
all in organic diseases of the heart. Baumgarten has published 
a comprehensive study treating of this condition (Disturb- 
ances of the Heart Rhythm with Reference to their Causa- 
tion and their Value for Diagnosis, Transact, of the Assoc. 
of American Physicians, 1888). Rummo and Ferranini have at- 
tempted to investigate this condition experimentally (Riforma 
med., December, 1887, 278-287), but much is still obscure. 

C. The Disturbances of the Digestive Organs due to 
Lesions of the Vagus. 

The vagus forms two gastric plexuses: the one, the anterior, 
situated on the anterior surface, and the other, the posterior, situ- 
ated on the posterior surface of the smaller curvature of the stomach. 
The first plexus is formed by the left, the second by the right, a 
somewhat stouter nerve. The branches of these plexuses associate 
with fibres from the sympathetic which accompany the ramifications 
of the coronary arteries; a part of the fibres which appertain to the 
right (posterior) vagus go on to the cceliac plexus, and can in a 
careful dissection be traced to the spleen, the liver, the kidneys, and 
small intestine. 

The muscles of the oesophagus and stomach are also innervated 
by the vagus ; its sensory fibres conduct the impulses concerned in 
the reflex actions of deglutition, sobbing, and vomiting. 

Among the disorders of the digestive organs caused by dis- 
ease of the vagus, the so-called stomach and intestinal neuroses, 



DISEASES OF THE VAGUS {PNEUMOGASTRIC NERVE). j$i 

we find affections of the motor, sensory, secretory, and perhaps 
also of the trophic fibres. Among the motor neuroses we have, 
according" to Glax (Klin. Zeit- und Streitfragen, 1887, i, Heft 
6), irritative and depressive forms. The former manifest them- 
selves in simple peristaltic unrest of the stomach, or in nervous 
belching or vomiting, the latter in nervous atony of the stom- 
ach, or insufficiency of the cardia or pylorus. Merycism, or 
rumination, must also be classed among the motor neuroses. 
Among the sensory disorders we find cardialgia and hepatal- 
gia. Of the secretory neuroses, nervous dyspepsia is the most 
important. To this class also belongs, in all probability, the 
so-called cesophagismus. The claim of Arndt (Deutsche med. 
Wochenschr., 1886, xiv, 5) that the round ulcer of the stomach 
should be regarded as " originating in a neurotic affection, an 
angio- or tropho-neurosis (of the vagus)," is deserving of further 
investigation. 

These vagus neuroses are rarely met with alone in other- 
wise healthy persons ; more often they appear in conjunction 
with other diseases, especially general affections of the nervous 
system, particularly hysteria or tabes. Sometimes they are 
associated with affections of the uterus, such as displacements 
(Panecki, Therap. Monatsh., 1892, 2); finally, they are met with 
in pregnancy. Possibly some have a reflex origin. According 
to Leva (Munch, med. Wochenschr., 1890, 20, 21) this is the 
case in merycism ; but here we also find anomalies in the secre- 
tion of the gastric juice, a circumstance which may be of astio- 
logical importance. In most cases of rumination which have 
been observed the patients have eaten copiously and rapidly 
and have overloaded their stomachs with imperfectly masti- 
cated food (cf. Alt, Berlin, klin. Wochenschr., 1888, 16, 27; 
Boas, ibid., 31 ; Jiirgensen, ibid., 46; also the above-mentioned 
article of Leva, and one by Singer in the Deutsch. Arch. f. 
klin. Med., 1892, li, Heft 4, 5, articles in which especially the 
relation of rumination and vomiting is discussed). 

The other motor neuroses of the stomach and intestinal 
tract will be discussed in the chapter on Hysteria. 

Cardialgia. 

Cardialgia (gastralgia, gastrodynia) is a disease of the sen- 
sory nerves which occurs mostly in paroxysms. Romberg, 
distinguishing two forms, assumed the one to be due to a 
hyperesthesia of the vagus branches going to the stomach 



132 



DISEASES OF THE CRANIAL NERVES. 



(" gastrodynia neuralgica"), the other to a hyperassthesia of the 
solar plexus (neuralgia coeliaca). There have been, however, 
cases coming under notice which can not be classed under either 
of these heads, and even more which do not permit of a deci- 
sion as to which of the two forms we are dealing with. 

The characteristic symptoms of gastrodynia are violent 
paroxysmal constricting pains, starting in the region of the 
stomach and radiating to the back ; the face becomes livid, the 
hands and feet cold, the pulse smaller and intermittent, and a 
feeling of unutterable anguish and distress takes possession of 
the patient. If in the presence of these symptoms careful ex- 
amination has excluded the existence of any organic stomach 
lesion — e. g., acute or chronic catarrh, gastric ulcer or tumor — 
if there is no evidence of gall stones, and the patient has pre- 
viously at times been subject to neuralgia in other parts of his 
body, we make our diagnosis with some amount of certainty. 
But in all cases this can only be done after careful and repeated 
examination before and after meals ; not uncommonly we find 
that pain, which is present while the stomach is empty, is re- 
lieved by the ingestion of food, and the patient states that 
uniform firm pressure on the epigastrium has often a beneficial 
alleviating effect, both conditions not generally observed in 
organic diseases of the stomach. 

In the treatment of these cases we must first of all endeavor 
to remove any primary cause, and in this connection mental 
and physical overstrain, excesses in venery, masturbation, or 
uterine affections, must be thought of. Besides the external 
application of blisters to the epigastrium, arsenic given for sev- 
eral weeks is to be recommended. During the attack morphine 
can often not be dispensed with. The diet has to be care- 
fully regulated, but not restricted ; on the contrary, it is ad- 
visable for the patient to take four or five times daily sub- 
stantial but easily digested food. 

LITERATURE. 

Sawyer, J. Clinical Lecture on the Treatment of Gastralgia. Lancet, August 
13, 1887. 

Of great practical importance is the so-called hepatalgia or nerv- 
ous biliary colic, which was first described by Andral in 1827, and 
which has been studied more recently by Frerichs, Fiirbringer, and 
Talma (cf. Pariser, Deutsch. med. Wochenschr., 1893, 31). This 
affection is usually seen in anaemic women ; it manifests itself in 



DISEASES OF THE VAGUS (PNEUMOGASTRIC NERVE). ^3 

paroxysmal pains, which are as severe as those of a true biliary colic ; 
they are, however, more restricted to the hepatic region, and never, 
even after recurring for years, lead to febrile inflammatory affections 
of the liver, the gall-bladder, or the gall-ducts (Fiirbringer). Anti- 
neurasthenic treatment is often of no avail. 

Nervous Dyspepsia. 

The disease known as nervous dyspepsia is an extremely 
common neurosis of the vagus, especially in females. It is 
characterized by a loss of appetite, painful sensations in the 
region of the stomach, frequent vomiting, and still more fre- 
quent belching ; besides these the patients generally suffer from 
other nervous symptoms — dull headache, vertigo, palpitation ; 
they are easily tired, complain of a lump in their throat (globus 
hystericus), at times have a voracious appetite, and obstinate 
constipation is seldom absent. The motor functions of the 
stomach are, as a rule, more or less disordered, and sometimes 
secretory anomalies are observed ; indeed, only rarely do both 
the motor and chemical functions remain intact (Herzog, 
Zeitschr. f. klin. Med., 1890, xvii, 3,4). In rare cases periodical 
spells of vomiting have been noted (twenty to thirty in the 
twenty-four hours), accompanied by acute circumscribed swell- 
ings of the skin (angio-neurotic oedema, Strubing, Quincke). 
Although the patients feel very poorly, their state of nutrition 
remains, nevertheless, for a long time remarkably good ; only 
in a few cases do we observe a rapidly increasing and marked 
anaemia. It is still doubtful whether the condition is essentially 
a disease of the peripheral nerves of the stomach or a general 
neurosis (neurasthenia dyspeptica, Ewald). We would refer 
the reader to a most interesting and comprehensive article 
which has been written on this subject by Leube (Berl. klin. 
Wochenschr., No. 21, 1884). 

In making our diagnosis we are brought face to face with 
no inconsiderable difficulties. The claim of Leube that we are, 
in the presence of the above-described symptoms, justified in 
thinking of nervous dyspepsia if a stomach-washing six to 
seven hours after the meal shows the stomach to be empty, 
has been opposed by Ewald and others. These have shown 
that, on the one hand, the stomach may be empty seven hours 
after a meal in cases of ulcer, and, on the other hand, may 
contain remains of food in nervous dyspepsia after the same 
time. To be sure, an increase of hydrochloric acid (hyper- 



134 



DISEASES OF THE CRANIAL NERVES. 



acidity) is a common condition in gastric ulcer. The results 
of stomach-washing are, however, certainly not always pathog- 
nomonic, but we must rather for the purpose of diagnosis take 
into account the course of the disease and the general condition 
of the patient. Bat in spite of the greatest care experienced 
men not seldom in these cases are led into error. Under cer- 
tain circumstances the hyperemesis nervosa, a motor neurosis 
of the stomach occurring in pregnant women, especially in the 
first months of pregnancy, may closely simulate the disease. 

In the treatment our attention has chiefly to be directed to 
the proper nutrition of the patient. Of medicines, arsenic, 
quinine, chloral (i.o (grs. xv) several times a day), should be 
resorted to. Saline purgatives, a course of treatment at Carls- 
bad, as well as the use of electricity, are of no avail. A stay 
in the mountains, hydrotherapy, sea-baths, all should be tried 
in succession, and last, but not least, the possibilities of psy- 
chical treatment must not be forgotten. 

LITERATURE. 

Cherchewsky. Contributions a la pathologie des nevroses intestinales. Revue 
de med., 3, 1884. 

Schiile. Arch. f. Psych, u. Nervenkrankheiten, xv, 3, 828, 1884. (Nervous Dyspep- 
sia, with a Reflex Vagus Neurosis as a Complication. — Respiratory Difficulty.) 

Allbutt. Visceral Neuroses. Lancet, i, 11, 12, 14, 1884. 

Ihring. Die nervose Dyspepsie und ihre Folgekrankheiten. V. Volkmann's 
Sammlung klin. Vortrage, No. 283. 

Neustab. Neuroses of the Stomach. Russ. Med., 26-28, 1887. 

CEsophagism us. 

Spasmodic dysphagia, known as cesophagismus (spasm of 
the gullet), is an affection which sometimes follows dyspeptic 
symptoms and protracted vomiting, sometimes irritation of the 
fauces by hot food, irritating substances (mushrooms, red pep- 
per, etc.). Sometimes the spasm is seen to occur refiexly in 
consequence of uterine diseases, and quite frequently in hys- 
teria. As an independent affection it is rarely ever observed. 
In all cases it is characterized by the fact that the patient from 
time to time (periodically) finds it difficult, or is even un- 
able, to swallow his food ; that when it reaches a certain 
point it is regurgitated, and that the sound which is intro- 
duced for the purpose of examination is stopped at the same 
place ; if this point is situated in the upper portion of the 
oesophagus, usually violent pain is experienced on the inges- 



DISEASES OF THE VAGUS (PNEUMOGASTRIC NERVE). ^5 

tion more especially of cold food, a circumstance which makes 
the patient object to taking his nourishment, and consequently 
leads to emaciation, although the loss of flesh is here consider- 
ably less than in stenosis of the oesophagus caused by new 
growths, because in the former case the patient is able at times 
to swallow his food without any difficulty. 

Predisposed to cesophagismus are nervous, easily excitable^ 
hysterical persons, in whom the affection often suddenly makes 
its appearance after some emotion without the previous exist- 
ence of any symptoms referable to the oesophagus. It has 
often followed the suppression of the menses, or has appeared 
during pregnancy and lactation. Sometimes no other aetio- 
logical factor could be discovered than injuries to the gullet 
years previous to the spasm — burns, injury by sulphuric acid, 
etc. No definite statement is warranted as to the duration and 
the course of the disease, as both vary greatly, but this much 
may be said with certainty, that in pure cases the prognosis is 
always good, that complete recovery is almost always effected 
by the repeated use of the sound and by the application of the 
faradic brush. 

LITERATURE. 

Chamaillac. Journ. de med. et de chir. prat., p. 311, 1846. 

Matthieu. Gaz. med. de Lyon, p. 102, 1852. 

Gendron. Arch, gener., 5me sen, t. xi, p. 293, 1858. 

Vigla. Gaz. des hop., Septembre 25, 1869. 

Axenfeld. L'Union, 73, 1872. 

Hulke. Transact, of the Clin. Society, vol. vi, 1873. 

Roux. These de Paris, No. 105, 1873. 

Smith. Dubl. Quart. Journ., March, 1864. 

Peter. Gaz. des hop., 85, 1875. 

Mackenzie, Morell. Med. Times and Gaz., October 21, 1876. 

Eloy. Contribution to the Knowledge of CEsophagismus. Gaz. hebd., 2me 

serie, t. xvii, 46, 47, 50, 1880. 
Striibing. Ueber acutes angioneurotisches CEdem. Zeitschr. fur klin. Med., Bd. 

ix, 5, 1885. 
Meltzer. Ein Fall von Dysphagie nebst Bemerkungen. Berl. klin. Wochenschr., 

8, 1888. (Symptoms of (Esophagus-Stenosis lasting for Nineteen Years.) 
Lewin. Beitrage zur Pathologie des Vagus. Inaug.-Dissert., Petersburg;, 1888. 
Edinger. Vagusneurosen. Reprinted from Eulenburg's Real-Encyclopadie, 

2. Aufl. 
V. Schlern. Zur Behandlung der nervosen Magenkrankheiten. Berl. klin. 

Wochenschr., 1891, 20. 
Holm, Harald. Den dorsale Vaguskarnes Anatomi og Pathologi. Norsk Ma- 

gazin f. Laegevidensk., 1892, p. 1. 
Buchholz, E. Beitrage zur Kenntniss der Vagusneurosen. Inaug.-Dissert.-, 

Berlin, 1893. 



CHAPTER IX. 

THE DISEASES OF THE ACCESSORY NERVE. 

The accessorius consists of two parts, both of which have a sepa- 
rate origin and exit. The upper one belongs entirely to the vagus, 
emerges with it, and is hence called accessorius vagi. The lower one 
begins at the level of the first cervical nerve (cf. Fig. 26), and can be 
traced as far down as the level of the sixth, sometimes even of the 



co. a. 



.a.C.II 




Fig. 26. — Cross-section through the Cervical Cord. r.p. C. II, posterior root of 
the second cervical nerve. XI, fibres of accessorius. C. a., anterior horn. C.p., poste- 
rior horn. CI, lateral horn. H\ Goll's column. H*, Burdach's column. iS", lateral 
column. V, anterior column, g, substantia gelatinosa of posterior horn. 

seventh cervical nerve roots ; this is the spinal portion, the accesso- 
rius spinalis. After having passed upward to the foramen magnum, 
close to the cord, it unites inside the skull with the portion belong- 
ing to the vagus to form for a while a common trunk, the accessorius 
communis, which, soon after leaving the skull through the jugular 
foramen, again divides into two branches, the accessorius vagi then 
becoming the inner, the accessorius spinalis the outer branch. 
136 



THE DISEASES OF THE ACCESSORY NERVE. i^n 

The nucleus of the former portion has been described in the 
two previous chapters; that of the spinal portion is in the region 
of the anterior horn of the cervical cord. Since the latter is pro- 
vided with motor cells, the purely motor nature of the spinal por- 
tion is evident (Schwalbe). According to Dees (Allgem. Zeitschr. 
f. Psychiatrie von Laehr, Bd. 43, Heft 45, 1887), the nucleus of Ihe 
accessorius is divided into three portions, the upper being situated in 
the centre of the anterior horn, just above the first cervical nerve; 
the middle at the lateral border of the anterior horn from the sec- 
ond to the fourth cervical nerve; and the lowest at the base of the 
lateral horn from the fourth to the sixth cervical nerve. The large 
multipolar (motor) nerve cells which form the nucleus are arranged 
like a rosary. 

We may have central as well as peripheral diseases of the 
accessorius, and, as is the case in other motor cranial nerves, 
the diseases may be of a paralytic or of an irritative nature 
(hyperkinesis, spasm — akinesis, paralysis). 

Accessorius Spasm, Spasmodic Wryneck, Spasmodic 
Torticollis {Tic Rotatoire, Nickkrampf). 

Since the accessorius supplies the sterno-cleido-mastoid and 
the trapezius (with its posterior larger portion), it is these two 
muscles which present disturbances in affections of the nerve. 
Either of them may be affected by itself, by a clonic or a tonic 
form of spasm ; hence there exist quite a variety of clinical 
pictures, especially as the disease may also be unilateral or bi- 
lateral. The sterno-cleido-mastoid is about as often the seat 
of a clonic spasm as the trapezius, whereas the tonic form is 
very rarely seen in the latter muscle. 

By the rhythmical contractions of one sterno-cleido-mastoid 
the head is moved to one side in a very characteristic manner ; 
the chin is turned toward the opposite (well) side and is ele- 
vated, while the ear is approached to the clavicle. Contracture 
of this muscle (the tonic spasm) fixes the head in this position 
— caput obstipum spasticum. If both sterno-cleido-mastoids 
are affected, the head is drawn alternately first to the one, then 
to the other side (clonic form), or it is pulled strongly forward 
and bent toward the chest (tonic form of the spasm). 

Contractions of the trapezius draw the head backward and 
toward the diseased side, elevate the shoulder, and approach 
the scapula to the vertebral column. A tonic spasm in the 
same locality entails fixation of the head in this position. 



138 DISEASES OF THE CRANIAL NERVES. 

A simultaneous spasm of the sterno-cleido-mastoid and the 
trapezius of the same side, in which the facial muscles also 
sometimes take part, is known to occur more frequently than 
an affection of both trapezii or of both sterno-cleido-mastoids 
alone. The directions of the movements and the positions 
which result from such spasms can be made out from what has 
been just said. 

The occurrence of such affections is either in paroxysms or 
else we have permanent contractions, only ceasing or abating 
during sleep. Recovery is exceptional. All therapeutic meas- 
ures, not excluding the electrical and chirurgo-orthopaedic 
treatment, are usually unsuccessful. The thermo-cautery may 
be tried. Any internal medication would have to be con- 
ducted according to the principles described in the treatment 
of facial spasm. 

The causes of the disease differ widely. Cerebral tumors, 
meningitis, foci of softening, as well as caries of the cervical 
vertebras, new growths in the medulla oblongata, may give rise 
to central, while external influences, cold, etc., may give rise to 
peripheral affections of the nerve. There are, moreover, well- 
authenticated cases on record of reflex spasm in the distri- 
bution of the accessorius arising from irritation by worms, 
uterine trouble, fright, and other emotions. As a rule, no 
aetiological factor can be detected. An epileptic who came 
under my observation, a single woman, twenty-seven years of 
age, presented at times a spasmodic torticollis, the contractions 
being extremely violent, sometimes lasting for weeks, and again 
being almost entirely absent for the same length of time. 

Accessorius Paralysis. 

This very rare affection may take in one or both of the 
above-named muscles. Unilateral paralysis of the sterno-cleido- 
mastoid produces a wry position of the head, in which the chin 
is somewhat elevated and directed toward the diseased side. 
Turning of the head is difficult but not impossible, as other 
muscles are brought into play. Bilateral paralysis of the muscle 
causes the head to be held straight, and is characterized by the 
absence of the prominence which the normal muscle produces. 

Unilateral paralysis of the trapezius allows the scapula to 
sink downward, causing the distance between its internal mar- 
gin and the vertebral column to become greater. As a con- 
sequence, the arm falls forward, the clavicle becomes more 



THE DISEASES OF THE ACCESSORY NERVE. ■ 139 

prominent, the supraclavicular fossa more marked, and the 
posterior upper angle of the scapula can be distinctly felt. 
Voluntary elevation of the shoulder and the motion of the 
scapula toward the spinal column is interfered with, and be- 
comes only possible with the aid of the levator anguli scapula? 
and the rhomboidei. The arm can not well be raised above 
the horizontal position in spite of the serratus which acts nor- 
mally, because the action of the upper third of the trapezius is 
lost. The paralysis of both trapezii allows both shoulders to 
sink outward and forward, so that the back appears more 
curved ; the ability to support the head in the upright posture 
is sometimes interfered with. Simultaneous paralysis of the 
sterno-cleido-mastoids and the trapezii gives a combination of 
both clinical pictures. If, in addition, the inner (smaller) por- 
tion of the nerve takes part, the resulting paralysis of the 
laryngeal muscles, the velum palati, and the pharyngeal mus- 
cles manifests itself by hoarseness, the nasal tone of the voice, 
and difficulty in swallowing. The increase in the frequency of 
the pulse, which has in such cases been observed by Seelig- 
miiller, must be attributed to the simultaneous affection of the 
cardiac branches of the vagus. Prognosis and treatment are 
the same as in the spasmodic affections, and little more can be 
said about the aetiology. These forms of paralysis have been 
known to occur in consequence of certain occupations, e. g., in 
water-carriers (Seeligmuller), and in the course of tabes it has 
been seen as a bulbar affection. We may also imagine an injury 
to the nuclei of the nerve during difficult labor, and if we find 
symptoms of paralysis in the muscles of the neck in new-born 
children, who for the first few years of life are unable to hold 
the head straight, such a possibility ought not to be forgotten 
(Gowers). 

LITERATURE. 

Seeligmuller. Arch. f. Psych., iii, 2, p. 433, 1872. 

Kropff. Beobacht. liber Krampfe im Bereiche des Accessorius und der oberen 

Cervicalnerven. Diss. Inaug., Gottingen, 1875. 
Remak. Doppelseitige Lahmung des Accessorius Willisii. Deutsche med. 

Wochenschr., 27, 1885. 
Duchenne Wernicke. Physiologie der Bewegungen, pp. 588 et seq. Cassel u. 

Berlin, 1885. 
Marthas. Berliner klin. Wochenschr., No. 8, 1887. 
V. Keller. Deutsche med. Wochenschr., 1887, xiii, 27. 
Landolt. Torticollis ocularis. Bull, med., 1890, 50. 
Guibert. Crampe fonctionelle du cou. Revue de med., 1892, 4. 



CHAPTER X. 

DISEASES OF THE HYPOGLOSSAL NERVE. 

The ten to fifteen bundles of fibres of which the hypoglossal 
nerve consists, as it emerges from the medulla oblongata in the 
groove between the anterior pyramid and the olivary body, unite to 
form two larger bundles, which leave the dural space separately, and, 
after their entrance into the hypoglossal canal, the anterior condyloid 
foramen, become a single stem, which leaves the cranial cavity by 
this canal. Outside the base of the skull it passes along to the 
mesial side of the vagus, at first obliquely downward and forward, 
then obliquely upward, runs on the outer surface of the hyoglossus 
muscle, and soon reaches the region where the genioglossus muscle 
radiates into the tongue. There are various communications be- 
tween the hypoglossus, the vagus, the anterior branches of the upper 
cervical nerves, and the lingual branch of the trigeminus. 

The cortical area of the hypoglossal nerve is found, according to 
Exner, in the lower portion of the anterior central convolution and 
the adjoining portion of the inferior frontal convolution, as shown in 
Fig. 28. Its nucleus is situated in the floor of the fourth ventri- 
cle, where its very large nerve cells, which measure up to 60 p in 
diameter, closely resemble the large multipolar cells of the ante- 
rior horn in the cord. After the closure of the central canal it is 
situated to the ventral side of the latter. 

The root fibres of the hypoglossus certainly arise in part from 
the nucleus of the same side. To what extent the nucleus of the 
opposite side, as well as the group of nerve cells situated in its 
neighborhood and the above-mentioned nucleus ambiguus, can be 
considered sources of origin for them, and, moreover, whether direct 
fibres of the hypoglossus have their origin in the cerebrum, is still 
undecided. 

While in certain of the cranial nerves — for instance, in the 
facial — peripheral affections occur at least as frequently as cen- 
tral, in the case of the hypoglossal this is not true. Often as 
its nuclei take part in the most diverse diseases, especially of 
the cord and medulla oblongata, it is rare that a peripheral 
140 



DISEASES OF THE HYPOGLOSSAL NERVE. 



I 4 I 



affection comes under observation. That, in a given case, the 
disturbance is peripheral and not central, more especially not 
bulbar, we may conclude from the absence of other symptoms 
of bulbar disease, and from the possible presence of complete 




Fig. 27.— Superficial Origin of the Cranial Nerves. I-XII, the twelve cranial 
nerves. CI, anterior root of the first cervical nerve, ca, anterior column of the spinal 
cord, c/, lateral column, pa, anterior pyramids. <?, olivary body. P. V., pons Varolii, 
z', internal geniculate body, e, lateral geniculate body, tc, tuber ciner. /z, pituitary 
body. P, cerebral peduncle. Sy, region of the fissure of Sylvius, a, corpora albicantia. 
C, island of Reil. 7Vz, optic thalamus. 

reaction of degeneration, as Erb (cf. lit.) has done in his recent- 
ly described case. The symptoms otherwise are the same as 
in the central disease. 

Central paralysis of the hypoglossus may be, in the first 
place, of cortical origin. According to Exner, as has been 
stated, the cortical area for the tongue is situated close to the 



142 



DISEASES OF THE CRANIAL NERVES. 



point where the middle and inferior frontal convolutions join 
the anterior central convolution, and it is very probable that 
injury to this region causes a motor disturbance in the tongue. 
In a case of tubercular meningitis, Matthes (Miinchener med. 
Wochenschrift, 1892, 49) has observed a hypoglossal paralysis, 

Fissure of Rolando 

Parietal lobe 

Parieto-occlpilal 
ssurc 




Fig. 28.— Cortical Centres of the Left Hemisphere. (After Gowers.) 



which he attributes to a localized tubercular meningitis at 
the convexity over the centre for the hypoglossus. On the 
whole, central palsies of this nerve are rare. 

The bulbar lesion of the nerve, or rather of its nucleus, is 
somewhat better understood ; it has undoubtedly been ob- 
served, if not frequently, at least repeatedly, that this lesion 
can occur unilaterally. There is then an atrophy of the nu- 
cleus, in which the nerve cells and the medullated fibres be- 
come decreased in number or disappear entirely, while the 
roots appear as fine threads. In such cases (see especially Fig. 
29) the tongue is protruded, not straight, but deviates toward 
one side, and be it remembered toward the affected side (m. 
genioglossus and geniohyoideus) ; it shows fibrillary twitch- 
ings, and an atrophy of the diseased side — hemiatrophia lingua? 
— which in such a case looks flabby and shrunken in compari- 
son with the full and firm healthy half; it is wrinkled, con- 
tracted, and much smaller than the latter (cf. Figs. 29 and 30, 
showing my two cases). The electrical examination shows 
either normal reaction or reaction of degeneration; that the 



DISEASES OE THE HYPOGLOSSAL NERVE. 



J 43 



latter may also occur in central lesions has been demonstrated 
by one of my cases, which, however, did not come to autopsy. 
Speech, mastication, and deglutition often suffer considerably ; 
on the other hand, the healthy half of the tongue may develop 
so satisfactory and vicarious an activity that little disturbance 
is observable. 

Unilateral paralysis of the hypoglossal nerve, due to periph- 
eral causes (Birkett, Neurol. Centralblatt, 1891, 24) has been ob- 




Fig. 29. — Hemiatrophia Lingua (personal observation). 



served as a result of traumatism ; further, also, in diseases of 
the vertebral artery, as the result of new growths in the me- 
dulla oblongata and in cases of embolic softening in the region 
of the nucleus (Hirt). Whether it can be also of saturnine 
origin seems to me to be doubtful, in spite of the report of 
Remak. In a recently published article by Koch and Marie 
(cf. lit.) may be found all the cases observed up to the present 
time collected and minutely analyzed. A case of congenital 
hypoglossal paralysis has been observed by Francotte (Annal. 



144 DISEASES OF THE CRANIAL NERVES. 

de la soc* med.-chir. de Liege, 1889), which is undoubtedly an 
instance of infantile nuclear degeneration (Mobius). 

In bilateral paralysis of the hypoglossal the tongue, atrophic, 
wrinkled, and shrunken, lies almost motionless on the floor of 
the mouth ; the patient can not protrude it, and has entirely 
lost control over it. Speaking and chewing are rendered diffi- 
cult, even quite impossible. This sad picture is seen not infre- 
quently in Duchenne's progressive bulbar paralysis, occasionally 




Fig. 30.— Hemiatrophy Lingua (personal observation). 

in progressive muscular atrophy, very rarely in tabes. The 
hemiatrophy of the tongue, too, occurs much less frequently in 
the course of tabes than, to judge from the communications — 
for instance, those of Ballet (cf. lit.) — would seem to be the 
case. 

The peripheral form of the affection may yield to electrical 
treatment (faradization and galvanization); the central, so far 
as we know at present, is not amenable to any treatment. 

Hypoglossal spasm occurs sometimes unilaterally, some- 
what more frequently bilaterally. It is an exceedingly rare 



DISEASES OF THE HYPOGLOSSAL NERVE. 



145 



affection, in regard to which there have been but few good 
publications. There is a paroxysmal, involuntary spasm of the 
tongue, by which it is protruded and retracted, rolled violently 
around in the mouth, and so roughly pressed against the teeth 
that it may be quite severely injured. In some instances there 
occur short rhythmical twitchings in the whole tongue which 
disappear at times. Berger observed an aura before such an 
attack, which consisted in a sensation of tension and swelling 
of the tongue. In Dochmann's case the attacks occurred espe- 
cially at night, and were so violent that the patient was awak- 
ened from her sleep by the sudden spasmodic protrusion of the 
tongue. In one of my own cases the muscles of mastication 
took part in the affection in such a way that before the actual 
hypoglossal spasm occurred, the lower jaw was for half or a 
whole minute spasmodically jerked to and fro, up and down. 
After these movements had ceased the mouth remained half 
open, and the turning and rolling movements of the tongue 
commenced and lasted for about one minute. These attacks 
recurred ten to twenty times a day ; they came on for the first 
time three days after an epileptic fit, and have lasted unaltered 
ever since (for three years). The patient is otherwise perfectly 
healthy, and has a good family history. The pathogenesis of 
the disease, its anatomical seat (irritation of the hypoglossus 
centre? cortical or bulbar?), is obsCure. As an accompanying 
symptom of chorea and hysteria it is by far more common than 
as an independent affection. Possibly the so-called auctioneer's 
spasm (Zenner, Berliner klin. Wochenschrift, 1887, 17), which 
is caused by overexertion (speaking and shouting), should be 
classed as a form of hypoglossal spasm. The treatment is the 
same as in paralysis of the tongue. 

LITERATURE. 
I. Paralysis of the Hypoglossal A T erve. Hemiatrophia lingua. 
Fairlie-Clarke. A Treatise on the Diseases of the Tongue. London, 1873. 
Moser. Beitrag zur Diagnostik der Lage und Beschaffenheit von Krankheits- 

herden der Oblongata. Deutsches Arch. f. klin. Med., xxxv, p. 418, 1884. 
Ballet. De l'hemiatrophie de la langue. Arch, de Neurol, vii, 20, 1884. 
Hirt. Ueber Hemiatrophie der Zunge. Berl. klin. Wochenschr., No. 25, 1885. 
Erb. Ein seltener Fall von atrophischer Lahmung der N. hypogl. Deutsches 

Arch. f. klin. Med., xxxvii, p. 265, 1885. 
Schiffers. De l'hemiatrophie de la langue. Revue mens, de laryngologie, 

d'otologie, et de rhinologie. 
Remak, E. Ueber saturnine Hemiatrophie der Zunge. Berl. klin. Wochen- 
schr., xxiii, 25, 1886. 
10 



1 46 DISEASES OF THE CRANIAL NERVES. 

Sauer. Fall von traumat. Hypoglossus- u. Accessoriuslahmung. Inaug. Diss., 
Gottingen, 1886. (Unilateral Luxation between Atlas and Epistropheus.) 

Peel. Berl. klin. Wochenschr., No. 29, 1887. (Hemiatrophy of Tongue, with 
Left-sided Recurrens Paralysis.) 

Koch et Marie. Hemiatrophie de la langue. Revue de med., viii, 1, 1888. 

Morison. Brit. Med. Journ., July 14, 1888. (Unilateral Paralysis of the Hypo- 
glossal in Consequence of Traumatism.) 

Limbeck. Prager med. Wochenschr., 1889, 16. 

Pasquier et Marie. Semeiologie de la langue. Progres med., 1891, 12. 

Birkett. Neurol. Centralbl., 1891, 24. 

Lange, F. Ueber Zungenbewegungen. Arch. f. klin. Chir., 1893, xlvi, 3, 
p. 634. 

2. Hypoglossal Spasm. 

Berger. Ueber idiopathischen Zungenkrampf. Neurol. Centralbl., i, 3, 1882. 

Dochmann. Petersb. med. Wochenschr., 1, 1883. 

Wendt. Unilateral Spasm of the Tongue. Amer. Journ. Med. Sc, clxxvii, p. 

173, Jan., 1885. 
Erlenmeyer. Centralbl. f. Nervenheilk., ix, No. 5, 1886. (Case of Idiopathic 

Spasm of the Tongue.) 
Bernhardt. Ueber idiopathischen Zungenkrampf. Ibid., No. 11, 1886. 
Lange, F. Ein Fall von beiderseitigem idiopathischem Hypoglossuskrampf. 

Ein Beitrag zur Lehre vom Ritter-Rollett'schen Phanomen. Arch. f. klin. 

Chir., 1893, xlvi, Heft 4. 



CHAPTER XI. 

SIMULTANEOUS AFFECTION OF SEVERAL CRANIAL NERVES MULTIPLE 

PARALYSIS OF THE CRANIAL NERVES. 

After having thus considered the lesions of the individual 
cranial nerves, it remains for us to inquire under what condi- 
tions several of them may be simultaneously affected, and into 
the symptoms thus produced. According- to the observations 
collected up to the present time, an affection of this kind may 
have its seat in the peripheral or in the central course of the 
nerves, as well as in the cortical or nuclear centres. Only cer- 
tain of the affections of this latter kind are to be regarded as 
independent diseases, while the peripheral lesions are always 
only partial manifestations of other conditions. In rare cases 
a simultaneous peripheral lesion of several cranial nerves may 
occur in consequence of traumatism, operative interference, 
etc. A case in point, in a patient operated upon by Israel, has 
been published by Remak (Berl. klin. Wochenschr., 7, 1888). 
A carcinoma of the neck was extirpated, and by the operation 
the accessorius, the hypoglossus, and the sympatheticus were 
injured, or rather resected. The symptoms caused by the acci- 
dent were accurately described by Remak. Other instructive 
cases, due to traumatism, have been described by Mobius (cf. 

ut.). 

Among the general diseases in which multiple cranial nerve 
lesions may occur are chiefly tuberculosis and syphilis. 

Tubercular meningitis attacks, by preference, the mem- 
branes at the base, and implicates most of the cranial nerves 
emerging in that region, as we have seen in our account of the 
diseases of the meninges. Lately Kahler (cf. lit.) has again 
directed attention to the fact that, in consequence of syphilis, 
a peripheral neuritis of the cranial nerves sometimes develops, 
and that we may, besides general cerebral symptoms, have a 
progressive slow paralysis, which attacks one cranial nerve 

147 



1 48 DISEASES OF THE CRANIAL NERVES. 

after the other in irregular succession (cf. also Rothmann, 
Deutsche Med.-Ztg., 1893, 46). 

After diphtheria peculiar forms of paralysis are observed, 
which chiefly take in the muscles of the soft palate and the 
pharynx. Since these muscles are innervated by certain of the 
cranial nerves, and the disease is unquestionably — e. g., when 
the paralysis is unilateral — often of peripheral origin (central 
diseases can not in all other cases be excluded), we shall devote 
a few lines to the consideration of their nerve supply. 

The innervation of the palatal and pharyngeal muscles is by no 
means one of the clearest chapters in neurology. We do not know 
exactly which of the cranial nerves are concerned, nor their mode of 
distribution. Of the palatal muscles the levator palati is the most 
important. This receives motor fibres through the large superficial 
petrosal (of the trigeminus) from the spheno-palatine ganglion, which 
come from the facial and which also innervate the azygos uvulae. 
Whether or not, however, the vago-accessorius and the glosso-phar- 
yngeus are also concerned in the innervation of these muscles, as 
Gowers, for instance, seems to think, basing his arguments upon clin- 
ical observations, is not as yet decided. With regard to the pharyn- 
geal muscles, it is generally assumed that the stylo-pharyngeus and 
the middle constrictor are supplied by the glosso-pharyngeal nerve, 
and that the palato-pharyngeus, the superior and inferior constrict- 
ors, are innervated by the vagus. The participation of the accesso- 
rius is doubtful (Schwalbe). We see then that the nerves concerned 
in a paralysis of the pharynx are the facial, the glosso-pharyngeal, 
the vagus, possibly also the fifth and the accessorius. 

Pharyngeal paralysis may be either unilateral or bilateral. 
The unilateral form can only be diagnosticated if the patient is 
made to move the soft palate, for instance, in saying " Ah ! " 
While during rest it appears to be perfectly symmetrical, the 
base of the uvula deviates somewhat on motion towards the 
affected side, so that on that side a little way from the median 
line there is a slight depression not present on the well side ; 
sometimes also the soft palate is a little lower on the para- 
lyzed side even during rest. In the bilateral complete paralysis 
of the soft palate, the latter hangs down flaccidly and the uvula 
appears elongated ; on deep respiration and on phonation it 
remains motionless, and the reflex movements evoked by tick- 
ling the mucous membrane are lost. Speech becomes mark- 
edly altered, the voice acquires a nasal tone, due, of course, to 
the cavity of the nose not being shut off during phonation-; 



POST-DIPHTHERITIC PAPAL YSIS. 



149 



hence also the pronunciation of the explosive consonants 
"P" and "B" becomes impossible, owing- to the imperfect 




compression of the air ; they sound like " M." 
the anterior nares removes, as Duchenne has 
disability. From the same cause also 
fluids are regurgitated through the 
nose on attempts at swallowing, and 
deglutition in general becomes difficult. 

Recent examinations of the nerves (Arn- 
heim, Arch. f. Kinderkrankheiten, 1892, xiii ; 
and Hochhaus, Virchow's Archiv, 1892, cxxiv, 
Heft 2) have demonstrated that lesions are 
present in various peripheral nerves, not only 
those going to the muscles of the palate and 
the fauces. Hansemann also has described 
(Virchow's Arch., 1889, cxv, Heft 3) the condi- 
tion of the cranial nerves in diphtheria. Ab- 
sence of the knee-jerks has been repeatedly 
found associated with paresis of the palate in 
diphtheria (Bed. klin. Wochenschr., March 30, 
1885, p. 204). 

The prognosis in post-diphtheritic paralysis 
is not unfavorable if the velum palati alone is 
paralyzed. If, on the other hand, the muscles 
of the oesophagus also take part, the outlook 
becomes graver on account of the inability of 
the patient to take nourishment, and all the 
more so if feeding by the stomach-tube is not 
constantly and carefully practiced. If this is 
not done, aspiration pneumonia or inanition 
may bring about a fatal issue. 

The electrical treatment ought to be begun 
as early as possible. It consists in the direct 
faradization or galvanization of the velum and 
the frequent excitation of reflex movements of 
deglutition by stimulation of the throat. The 
uvula, the pillars of the pharynx, etc., are di- 
rectly touched and repeatedly stimulated by 
means of the curved button electrode (cf. Fig. 
31). The movements of deglutition are ob- 
tained if the anode is placed on the neck and 
the cathode (button electrode) is quickly drawn 



Closure of 
shown, this 




Fig. 31. — Pharyn- 
geal and Laryn- 
geal Electrode 
with Arrange- 
ment for making 
and breaking the 
Current. (After 
Erb,) 



i5o 



DISEASES OF THE CRANIAL NERVES. 



over one of the lateral surfaces of the larynx, six to ten cells 
sufficing- for the purpose. These gymnastics of the phar- 
yngeal muscles constitute an excellent remedy which can not 
be replaced by any other. It often leads rapidly to re- 
covery. 

Central diseases of several cranial nerves at the same time 
may also occur, and that, too, not only in their intracerebral 
course — which for but few of them is known, and for those 
only imperfectly — but also in the centres themselves. As a 
matter of fact, our knowledge about the centres situated in the 
cortex is also very incomplete, since we must again confess our 
comparative ignorance of the anatomy. Still, we shall not go 
too far if we assume that extensive cortical lesions may impli- 
cate several centres together, and there is no doubt but that 
they may be affected after or rather during an apoplectic 
attack by " indirect action." 

LITERATURE. 

Reinhard. Deutsche med. Wochenschr., 1885, No. 19. (Subcutaneous Injec- 
tions in the Region of the Neck of 0.001 (gr. -gL) of Strychnia daily.) 

Rothmann. Ibid., 1885, No. 52. (Paralysis of the Muscles of Respiration after 
Diphtheria.) 

Hansemann. Virchow's Archiv, 1889, cxv, Heft 3. 

Hallager. The Paralyses after Diphtheria. Hosp. Tid., 1890, 4. 

Garcia y Mansilla. Nature and Treatment of the Post-diphtheritic Paralyses. 
Rivista clin. de los hospitales, 1891, 32. 

Suckling. Brit. Med. Journ., May 28, 1892. (Three Cases of Paralysis of the 
Diaphragm after Diphtheria.) 

Of eminently practical importance are the nuclear affections 
of the cranial nerves. Referring the reader to the preceding 
chapters for the anatomical position of the individual nuclei, 
we will only remind him of the fact that these nuclei are situ- 
ated in the gray matter, partly of the mid- and 'tween- brain, 
partly in the medulla oblongata. The portion situated above 
the latter extends from the posterior wall of the infundibulum 
in the third ventricle to the level of the nucleus of the abdu- 
cens, and embraces the nuclei of the eye muscles (Wernicke). 
The other nuclei belong to the lower portion. 

Clinical observations now teach us that either of these por- 
tions may be affected by itself, and we may with Wernicke call 
the disease of the upper, polioencephalitis superior; that of the 
lower, polioencephalitis inferior. According to the course, we 
distinguish in either case an acute and a chronic form, so that 



POLIOENCEPHALITIS {WERNICKE). I5I 

there are altogether four clinical pictures of these nuclear 
affections. 

Polioencephalitis superior acuta has only been observed in 
very few instances. The best observations we owe to Wernicke. 
According to him, this is essentially an acute inflammatory dis- 
ease of the nuclei of the ocular muscles, and proves fatal in from 
ten days to a fortnight, the focal symptoms consisting in an 
associated paralysis of the eye muscles, the general symptoms 
being grave disturbances of consciousness. The walk presents 
a peculiar combination of spasm and ataxia. Anatomically, 
foci of acute softening are found in the region of the nuclei, 
which are either due to obstruction of the blood-vessels or 
to inflammatory infiltration of the tissues. ^Etiologically, the 
abuse of alcohol may be mentioned. 

With reference to the diagnosis, the presence of a tumor in 
the region of the corpora quadrigemina should be considered 
(B. Sachs, New York, Diseases of the Mid-brain Region, Am. 
Jour, of the Med. Sci., March, 1891). 

Polioencephalitis superior chronica was described in 1868 
by von Graefe, and called by him ophthalmoplegia progres- 
siva. The first published case presented, according to von 
Graefe (Berl. klin. Wochenschr., 11, 1868), a peculiar clinical 
picture : 

" Gradually all the muscles concerned in the movements of 
the eye become paralyzed, so that there results first a diminu- 
tion in the range of sight, and finally complete immobility of 
the eyeballs. The levator palpebral superioris is wont to be 
implicated, although the consequent ptosis is rarely as marked 
as that occurring in complete oculo-motor paralysis. It is re- 
markable that, on examination for reaction to light and accom- 
modation, the sphincter pupillae as well as the ciliary muscle 
present no changes. This condition, which we very rarely find 
in other extensive oculo-motor paralyses, seems here constant 
and characteristic of this disease. Another feature which dis- 
tinguishes this form from other associated paralyses in the dis- 
tribution of the third, fourth, and sixth nerves is the progress 
of the disease pari passu in the antagonizing muscles. Thus we 
never find a marked strabismus divergens owing to a dominat- 
ing oculo-motor paralysis, because here the external rectus loses 
its functions sufficiently to neutralize the tendency to devia- 
tion, and the sight of the patient is therefore, in spite of the 
associated paralysis of the eye muscles, affected much less than 



152 



DISEASES OF THE CRANIAL NERVE. 



in simple oculo-motor or abducens paralysis. . . . Still, a cer- 
tain degree of asymmetry in the affection of the different mus- 
cles of one eye, as well as in the development of the whole dis- 
ease in the two eyes, may at times be found." (Cf. Wernicke, 
loc. cit., vol. iii, p. 463.) 

With the exception of this associated ocular palsy, which, 
developing progressively, may remain stationary without being 
completely symmetrical, the patient enjoys good health and 
complains neither of headache nor of symptoms of increased 
intracranial pressure. In isolated instances bulbar paralysis 
has been known to be later superadded, and in others the dis- 
ease was found associated with multiple sclerosis or with gen- 
eral paralysis (Ballet, Progres med., 1893, 23). Anatomically, 
the affection depends either upon a primary disease of the 
nerve nuclei or upon a diffuse sclerotic process in which the 
nuclei take part. In exceptional cases, which in their na- 
ture are as yet entirely obscure, no organic changes what- 
ever have been found, although the clinical picture corre- 
sponded exactly to that described by von Graefe. (Eisenlohr 
and Oppenheim.) 

Not less interesting, and at the same time of far greater 
practical importance because relatively far more frequently 
met with, is the fourth and last of the affections under consid- 
eration — a disease the first accurate description of which we 
owe to Duchenne, of Boulogne, and which after him has been 
carefully and successfully studied by German investigators 
(Wachsmuth, Kussmaul, Leyden) — the chronic progressive 
bulbar paralysis. 

Progressive Bulbar Paralysis. 

Paralysis of the Tongue, the Soft Palate, and the Lips {Duchenne, i860), Glosso-labial 
Laryngeal Paralysis { Trousseau), Chronic Progressive Bulbar Paralysis { Wachs- 
muth, 1864), Atrophic Bulbar Paralysis {Leyden), Bulbar Nuclear Paralysis {Kuss- 
maul), Polioencephalitis Lnferior Chronica { Wernicke). 

Duchenne s Disease. 
Symptoms and Course. — In the majority of instances the 
onset of progressive bulbar paralysis is very gradual, and only 
rarely do we meet with cases in which it is ushered in by an 
apoplectiform attack. After having complained for weeks, 
perhaps months, of drawing, tearing pains in the neck and the 
back, the patient discovers of his own accord or from the re- 
marks of his relatives that the enunciation of certain words, 



BULBAR PARALYSIS. 



53 



especially those containing /, r, and long e, has become very dif- 
ficult. If he happen to use a word containing all these letters 
(for instance, reel), he becomes painfully conscious of his indis- 
tinct enunciation. In vain he attempts to repeat the trouble- 
some words over and over again in order to correct his mis- 
take. He only becomes more convinced that the movements 
of his tongue have become clumsy, and that he has lost his 
former ease and fluency of speech ; and, in truth, it is the on- 
coming paresis of the lingual muscles which is the main cause 
of the disturbance. 

The tongue, which can not be raised to the normal extent, 
can no longer be approached sufficiently to the hard palate, 
and thus the long e, for the pronunciation of which the move- 
ment is necessary, can only be pronounced with difficulty. In 
the same way all the finer muscular movements required for 
the formation of the Unguals are imperfect, and consequently 
the enunciation of these sounds is bad. The disease progresses 
and the articulation becomes worse and worse, the less per- 
fectly the lingual muscles are innervated, and other letters, 
s, t, g, finally also d and n, begin to suffer, so that conversation 
with the patient becomes very uncomfortable, as certain words 
are almost unintelligible and others at least difficult to under- 
stand. 

The lips also begin to do their duty badly, so that the enun- 
ciation of the so-called labials — o, u, a, b,p — gradually becomes 
indistinct. The presence of strangers with whom he has to 
converse excites the patient, and, avoiding all society, he pre- 
fers the quiet monotony of the family circle, where nobody 
seems to pay much attention to the change in his speech (" ala- 
lia and anarthria "). Moreover, a change in the features of his 
face, at first slight and only noticed by the patient himself, but 
later more perceptible and evident also to his friends, gradually 
manifests itself, which serves as an additional reason for 
seclusion (Fig. 32). When laughing, it appears to him as if 
a certain tension in his lips prevented the usual play of the 
mouth. In the attempt to whistle, the lips can not be puckered 
as well as formerly ; the muscles of the cheek have become 
more rigid and inactive, and as the disease progresses the 
whole lower half of the face assumes a characteristic appear- 
ance — a peculiar lachrymose and astonished expression — which, 
as is easily seen, is due to the drooping of the lower lip and to 
the deepening of the naso-labial fold. The upper half of the 



54 



DISEASES OF THE CRANIAL NERVES. 



face, the forehead and eyes, do not take part in the change, 
but remain entirely normal. Nevertheless the patient's face is 
much disfigured, and later on in the disease may have become 
almost unrecognizable. 

While thus quite gradually symptoms have arisen which 
make the patient a very pitiable object, and which are bound 
sooner or later to interfere with his position in society, the sad 




Fig. 32.— Facial Expression in Progressive Bulbar Paralysis. (After 
Leyden, Eichhorst.) 



truth dawns upon him that even the functions absolutely neces- 
sary for the existence of life are failing. Eating, in which up 
to this time no trouble was experienced, he now finds difficult. 
It takes a longer time to swallow the food, and in a later stage 
even mastication becomes impaired. Not only do the move- 
ments of the lower jaw become weaker and less energetic, 
owing to paresis of the muscles of mastication, but, since the 
powerless tongue is unable to get the food from between the 



BULBAR PARALYSIS. 



155 



cheeks and gums into the region of the pharyngeal muscles, 
the formation of the bolus is impossible. Spoons, fingers, and 
the like, have to be used instead, or the patient has to hold his 
head far back to get the food to slide down. Even drinking 
causes much discomfort, as the liquid may get into the larynx 
and thus give rise to violent coughing, or may be regurgi- 
tated through the nose, either condition being due to weak- 
ness of the pharyngeal and laryngeal muscles. 

The implication of the larynx is very distressing, and may 
indeed become dangerous. The voice at times fails, speech 
becomes irksome, and the tone is monotonous ; production of 
the higher notes as in singing becomes impossible ; later on a 
marked hoarseness and finally aphonia follow, so that the pa- 
tient can only express himself in whispers, which, owing to the 
above-described motor changes, are quite unintelligible. At 
the same time the absence of a firm closure of the glottis, and 
therefore the inability to cough forcibly, gives rise to various 
disturbances in the respiratory apparatus, owing to the dis- 
ability to dislodge mucoid masses which may have collected in 
the air passages. 

Another symptom which, though not constant, is frequently 
met with, is the marked increase in the secretion of the saliva. 
This occurs usually rather early in the disease, and not infre- 
quently such patients are seen going around constantly holding 
their handkerchiefs to the mouth to prevent the saliva from 
trickling away. On examination, the secretion is found to be 
viscid. This flow of saliva is due to an actual increase in the 
amount secreted, as several careful investigators have shown, 
though they do not agree as to the exact amount. 

Two, three, even five, years may pass before any new symp- 
toms are added to those just described. These, however, pro- 
gressively gain in intensity, and it is especially the change in 
the features which becomes more accentuated, owing to the 
constantly increasing atrophy in the muscles of the lips and the 
cheeks ; the palatal reflexes become markedly decreased and 
finally lost; the tongue, shrunken and distinctly smaller, lies 
immobile on the floor of the mouth, and can neither be pro- 
truded nor moved in any direction. Fibrillary tremor is then 
not uncommonly marked. On the electrical examination 
(which is, by the way, very hard to make), we may find reac- 
tion of degeneration in the lingual as well as in the pharyngeal 
muscles. 



1 5 6 



DISEASES OF THE CRANIAL NERVES. 



The inability to take food properly is usually the cause of 
death ; the patient pines away, and gradually dies from inani- 
tion without having- the blissful benefit of a dulled conscious- 
ness to guide him insensibly through his tormenting sufferings. 
Only in occasional instances disease of the respiratory organs, 
caused by aspiration of food, hastens the termination (aspira- 
tion pneumonia). 

Pathological Anatomy. — There is hardly another disease of 
the nervous system with the anatomical basis of which we are 
better acquainted than bulbar paralysis. Duchenne pronounced 
the process to be a primary pigmentary degeneration, and 




Fig- 33- — Cross-section through the Upper Portion of the Medulla Oblongata. 
On the left the healthy, on the right the diseased medulla, hk on the left the normal, 
hk on the right the diseased hypoglossus nucleus (the nerve cells are almost entirely ab- 
sent on the right side). 



atrophy of the large nerve cells in the nuclei of the me- 
dulla oblongata, an assertion which has received complete con- 
firmation from all subsequent investigators. Microscopical 
examination shows atrophy of the nerve cells. This is shown 
in Fig. 33 in the nucleus of the hypoglossal ; the cells have 
in this case completely disappeared, having previously di- 
minished in size and lost their processes. At the same time 
we find the connective tissue increased, the walls of the vessels 
in the nucleus thickened. Similar changes are found in the 
nucleus of the vagus accessory and the glosso-pharyngeal 
nerves (the so-called lateral mixed system, cf. page 107). The 
former may become diseased in consequence of an ascending 



BULBAR PARALYSIS. 



157 



neuritis ; a myelitis may be caused by a similar process in the 
nerves of the lower extremities (Cupfer, Revue de med., 1890). 
Since, as we have said before, the upper part of the face always 
remains normal during the disease, we have to assume that the 
fibres innervating these muscles arise from a special centre. 
This is supposed to be a part of the abductor nucleus (Mey- 
nert), which has therefore come to be designated by the com- 
posite name of facial-abducens nucleus. This and the remain- 
ing nuclei, with the exception 
of those mentioned above, 
were always found to be in- 
tact. The atrophy also ex- 
tends to the root fibres, which 
to the naked eye often ap- 
pear smaller and of a grayish 
color. From the topograph- 
ical position of the nuclei be- 
low the floor of the fourth 
ventricle, as it is approxi- 
mately represented in Fig. 
34, we can easily understand 
how, on the one hand, the 
pathological process, after 
having attacked the hypo- 
glossals, next implicates the 
neighboring vagus, and, on 
the other hand, how the mo- 
tor part of the trigeminus 
usually remains unaffected, 
so that paralysis of the mus- 
cles of mastication is very 
rare. But why the auditory 
is constantly exempt and the 
facial partially affected are 
circumstances which need to be further investigated. A com- 
plete counterpart to bulbar paralysis is found in the so-called 
progressive muscular atrophy, a disease in which, as we shall 
see later on, the gray anterior horns of the spinal cord and 
their nerve cells are affected precisely in the same way as 
the bulbar nuclei in the disease we are now discussing. The 
nerve cells of the anterior horns constitute the trophoki- 
netic centres for the muscles supplied by the spinal nerves, an 




Fig. 34. — The Posterior (Dorsal) Aspect 
of the Medulla Oblongata, i, posteri- 
or commissure. 2, peduncle of pineal gland. 
3, corpora quadrigemina. 4, superior pedun- 
cle. 5, middle peduncle. 6, inferior pedun- 
cle of cerebellum. 7, stria? acusticas. 8, fu- 
nic, teres. 9, obex. 10, funicul. gracil. 



158 DISEASES OF THE CRANIAL NERVES. 

office which the bulbar nuclei fulfill for those supplied by the 
cranial nerves. In both diseases there are atrophy and decrease 
in the functional power, and in both the disturbance is strictly 
motor, while sensation is absolutely intact. This essential simi- 
larity between the two diseases explains why not rarely one is 
associated with the other — in other words, why they may com- 
plicate each other. We may, indeed we frequently do, meet 
with cases in which bulbar paralysis is accompanied by atro- 
phy of the muscles of the extremities, while, on the other hand, 
in progressive muscular atrophy, bulbar symptoms, disturb- 
ances in deglutition and speech, may be found. 

Another analogy exists between bulbar paralysis and amyo- 
trophic lateral sclerosis, a disease in which not only the nerve 
cells of the anterior gray horns, but also the motor tract 
in the lateral columns of the spinal cord are affected. All 
these diseases, viewed from an anatomical standpoint, if not 
identical, certainly are closely related to each other, and only 
differ in the position of the lesions ; it is therefore advisable to 
consider and study them from a common point of view, as the 
understanding of the individual symptoms will thus be much 
less difficult. 

Diagnosis. — As to the diagnosis, we need not be doubtful if 
we always remember that the disturbances are confined to the 
motor functions of the nerves governing the muscles of the lips, 
tongue, pharynx, and larynx. Oppenheim has recently called 
attention to rhythmical twitchings of the velum palati and of 
the internal and external muscular tissue of the lar}mx, which 
he considers to be of diagnostic value in diseases of the posterior 
fossa of the skull (Neurol. Centralbl., 1889, 5). If we find any 
well-marked sensory changes, if the patient complains of pain 
or paresthesias and the like, we either have to give up the 
diagnosis of bulbar paralysis, or we have to search for some 
complication. The peculiar facial expression, the increased 
flow of saliva, the tremulous atrophic tongue partially or even 
completely immobile as it is, the disturbance in speech and 
deglutition, when taken together are so characteristic that, if 
intelligently observed and studied, they will make our diag- 
nosis clear. 

There is only one case in which we may be doubtful ; cer- 
tain foci of disease in the brain may produce symptoms simu- 
lating bulbar paralysis, so much so indeed that the name pseudo- 
bulbar paralysis has been given to the condition (which later on 



BULBAR PARALYSIS. 



159 



will be described more at length) ; nevertheless, with due care- 
fulness we can avoid a mistake. The most important point to 
observe in the differential diagnosis is the course of the disease. 
While in progressive chronic bulbar paralysis this is slow, but 
always progressive toward the fatal end, in the spurious form 
remissions may occur, so that for years the patient may be im- 
proved, though he finally also succumbs to the disease. Besides 
this, pseudo-bulbar paralysis is often attended with cerebral 
symptoms, headache, apoplectiform attacks, etc. 

Prognosis. — The prognosis, as we should expect after what 
has been said, is altogether unfavorable. There is, according 
to our present knowledge, no cure for the true bulbar paralysis, 
and one ought to be careful, therefore, not to deceive the family 
with promises. As soon as the diagnosis is made they ought 
to be informed of the unfavorable outlook. 

Treatment. — The only treatment from which any success 
may be expected, if begun early, is the systematic use of elec- 
tricity : faradization and galvanization of the threatened mus- 
cles, especially of those of the tongue and pharynx, frequent 
excitation of the movements of deglutition, according to the 
method already described, are the only measures which deserve 
confidence. With the exception of this local treatment, there 
is nothing that affords even a temporary benefit. I have never 
seen any lasting effect from hydrotherapy, but still this treat- 
ment is very frequently advised just at that stage of the disease 
when electricity might do some good. Internal remedies are 
of no avail ; the occasional symptomatic use of atropine ( x / 2 to 1 
milligramme (y i20 to */& gr.) daily) to diminish the salivary secre- 
tion may be indicated. It scarcely needs to be mentioned that 
the chief duty of the physician in the later stages of the disease 
is to pay the most careful attention to the general nutrition of 
the patient. 

iEtiology. — The aetiology is still obscure. It is true that 
there are patients affected with the disease who, owing to their 
occupation, have made rather excessive use of the muscles of 
the lips, tongue, and palate (glass-blowers, musicians). These 
cases, however, are so rare that it would seem very forced to 
attribute any ^etiological importance to this factor. The same 
may be said about syphilis, the truth being that, in most cases, 
the cause is absolutely obscure, and all we can say is that males 
and persons advanced in life seem to be more frequently at- 
tacked by the disease than others. Heredity but rarely plays 



l6o DISEASES OF THE CRANIAL NERVES. 

a part, and the influence of cold remains, in connection with 
this disease, as obscure as with all other nervous affections. 

LITERATURE. 

Mobius. Ueber mehrfache Hirnnervenlabmung. Erlenmeyer'sches Centralbl. 

f. Nervenhk., 1887, x, 15, 16. 
Oppenheim und Siemerling. Die acute Bulbarparalyse und die Pseudobulbar- 

paralyse. Charite-Annalen, 1887, xii. 
Unverricht. Ueber multiple Hirnnervenlahmung. Fortschr. d. Med., 1887, 24. 
Pel. Berl. klin. Wochenschr., 1887, xxiv, 29. 
Mobius. Centralbl. f. Nervenhk., 1887, x, 15, 16. 
Adamkiewicz. Halbseitige fortschreitende Gehirnnervenlahmung. Wiener 

med. Wochenschr., 1889, 2. 
Scheiber. Berl. klin. Wochenschr., 1889, xxvi, 28. (Unilateral Bulbar Paral- 
ysis.) 
Reinhold. Deutsches Arch. f. klin. Medicin, 1889, xlvi, Heft 1. 
Mendel. Neurol. Centralbl., 1890, 16. 
Howard H. Tooth. Study of a Case of Bulbar Paralysis, with Notes on the 

Origin of Certain Cranial Nerves. Brain, 1891, 56. 
Senator. Acute Bulbarlahmung durch Blutung in der Oblongata. Reprint 

from Charite-Annalen, xvi, Jahrg. 
Senator. Bulbarlahmung ohne anatomischen Befund. Neurol. Centralbl., 

1892, 6. 
Remak. Berl. klin. Wochenschr., 1892, 44. 



PART III. 

DISEASES OF THE BRAIN PROPER. 

The more autopsies we see the more the fact is brought 
home to us that brain lesions are frequently present which 
were not diagnosticated during life. This is by no means 
necessarily the fault of the diagnostician, for undoubtedly 
many focal lesions of the brain may exist without giving rise 
to any symptoms. Recently G. Schmid has published an in- 
teresting collection of such cases (Virchow's Archiv, 1893, 
cxxxiv, 1). On the other hand, of course, we frequently see 
cases presenting symptoms which make us at once suspect the 
existence of a brain lesion. 

In such cases we have to ask ourselves two questions: (1) 
Where is the seat of the lesion? (2) What is its pathological 
nature ? To the physician both of these questions are of in- 
terest; to the patient, more especially the latter. 

The examination which searches for the seat of the lesion 
will give us the topical diagnosis (ro7ro5 = place) ; the exam- 
ination concerning the nature of the lesion, the pathological 
diagnosis. 

The endeavor to localize cerebral lesions — that is, to make 
a topical diagnosis — has only of comparatively late years re- 
ceived attention, and much of the work so far done can not be 
called more than an attempt, in many cases indeed only a weak 
one. The celebrated discovery of Broca (1861), that certain 
disturbances of speech were often found associated with lesions 
in the third left frontal convolution, the discovery of Fritsch 
and Hitzig(i87o) that stimulation of certain areas of the cortex 
produces contractions in certain definite groups of muscles on 
the opposite side of the body — these and various other, patho- 
logical, observations, to which reference will be made later, 
make it most probable, nay, almost certain, that definite parts 
or areas of the cortex are always connected with certain func- 
11 161 



1 62 DISEASES OF THE BRAIN PROPER. 

tions of the brain ; in other words, that these functions can be 
localized ; and, notwithstanding the many uncertainties and 
numerous contradictions between the results of experiments 
on the one hand and those obtained from clinical observations 
on the other, it is this doctrine of cerebral localization which, 
though still undeveloped, must be considered as the basis of 
all further investigation in the field of cerebral pathology. 

Equal stress must, however, be laid upon the examination 
into the nature of the lesion. A certain symptom — for instance, 
a persistent hemiplegia — is always the result of a lesion of the 
motor tract ; a lesion, however, which can be produced in 
quite a variety of ways. It may be due to cerebral haemor- 
rhage, to a tumor, an abscess, etc. It is therefore, especially 
with regard to the prognosis, of the greatest importance to 
determine the exact nature of the lesion in a given case, but 
both questions ought always to be investigated with equal 
care if we wish to arrive at as exact a diagnosis as circum- 
stances permit. In the following pages these two modes of 
diagnosis will be considered separately, and we shall first speak 
of what is known about cerebral localization, while in a later 
chapter the pathological side will be discussed. 

I. The Study of Cerebral Lesions with Reference to 
their Seat — Topjcal Diagnosis — Doctrine of Local- 
ization. 

Two classes of symptoms produced by cerebral lesions 
must be distinguished : first, general or diffuse (Griesinger), 
and, secondly, local. The former, so far as they concern the 
subjective feelings of the patient and the disturbances of the 
vegetative functions (temperature, pulse, respiration, condi- 
tion of urine), are to be observed and described in this con. 
nection in the same way as in diseases of other organs. The 
latter — the local symptoms — may be divided into two classes, 
namely, the direct and the indirect. We call those symp- 
toms direct which are produced by a persistent disturbance in 
the functions of a certain part of the brain. They are also 
called focal symptoms (Griesinger). By indirect symptoms we 
mean those which are only produced by transient conditions 
— changes in circulation, by compression, etc. — and which are 
in a way concomitant symptoms of the former. They may be 
entirely absent ; on the other hand, they may be so prominent 
as to make a topical diagnosis impossible. 



CEREBRAL LOCALIZA TION. 



163 



Destruction of a circumscribed area in the brain gives rise 
to symptoms of paralysis, or less frequently to symptoms of 
irritation. The former, where we have to deal with a loss of 
function, are also (after Goltz) called symptoms of destruction 
(Ausfallssymptome), and if the function is not lost but is only 
impaired, symptoms of impairment (Hemmungssymptome). The 
latter — namely, the irritative symptoms — are usually due to a 
so-called indirect action. 

It is not always possible to say whether a symptom is of a 
direct or of an indirect nature. For instance, if we find a pa- 
tient in an unconscious state with a hemiplegia, this hemiplegia 
may be a direct focal symptom or it may have been produced 
indirectly. In the latter case it will disappear in a few hours or 
days, in the former it will be persistent. Or, if a patient suffer- 
ing from the consequences of a cerebral haemorrhage presents, 
as is often the case, disturbances in speech, this may again 
be a focal symptom or not. If, after consciousness has been 
regained, the speech becomes gradually but steadily better, 
then the aphasia was produced indirectly. If, however, speech 
remains unintelligible for months or years, it is clear that we 
have to do with a focal symptom. Therefore, in acute lesions 
we can only after a certain time has elapsed discriminate be- 
tween direct and indirect symptoms. 

The irritative symptoms consist either of general epilepti- 
form convulsions or of partial, involuntary movements of the 
extremities (hemichorea, athetosis), of tremors, contractures, or 
forced movements of the whole body. We shall repeatedly 
have occasion to refer to these phenomena. 

Not all of the symptoms have an equal value for the lo- 
calization of a lesion. It is important first to note their mode 
of onset, whether this is sudden or gradual ; whether several 
symptoms have made their appearance at the same time or 
one after the other, and so on, for in acute lesions, for instance, 
only those symptoms which appear synchronously are of im- 
portance. If a patient who has a hemiplegia presents a paraly- 
sis of the oculo-motor of the opposite side, and we learn that 
this latter has existed before the onset of the hemiplegia, no- 
body certainly will think of connecting the two or look upon 
them as being symptoms due to one focal lesion. This would 
only be allowable if both had set in at the same time (after an 
acute lesion). 



164 DISEASES OF THE BRAIN PROPER. 

But, even apart from the mode of onset, the symptoms are 
not of equal value in the localization. Some, it is true, as 
hemiplegia, together with contra-lateral oculo-motor paralysis, 
are almost pathognomonic (for a lesion in the crus), and their 
simultaneous appearance is therefore extremely important ; 
while others, as the conjugate deviation in severe hemiplegia, 
are found in different lesions, and are therefore less significant; 
still others, as optic neuritis and all the general symptoms 
(headache, vertigo, unconsciousness), are absolutely valueless. 

We see, therefore, that by no means all cases can be used 
for the study of the topical diagnosis, but only those in which 
the affection, in the first place, remains chronically station- 
ary; secondly, in which it is circumscribed and isolated (Noth- 
nagel) ; and, thirdly, where the surroundings of the focus are 
as little as possible implicated. These three conditions are 
best fulfilled in instances of haemorrhage or embolus, or rather 
in the cases of softening produced by these accidents, and the 
largest contingent of cases which permit a topical diagnosis is 
therefore made up of these. They are rendered more suitable 
for our purpose the longer the time that has elapsed after the 
first onset (according to Nothnagel, six to eight weeks), as only 
then, as we stated above, are we able to separate the direct 
from the indirect symptoms. In other cerebral affections — e. g., 
meningitis, encephalitis, and especially tumors — a local diagno- 
sis should only be attempted with the greatest circumspection, 
and even then errors can not be altogether excluded. 

Symptoms Referable to Cortical Lesions. 

In speaking of cortical lesions, " surface lesions," it must 
not be forgotten that the clinical meaning of the term is differ- 
ent from the anatomical one. Anatomically, it implies that the 
medullary layer situated below the cortex is intact, while clin- 
ically we speak of cortical lesions even if the white matter 
takes part in the pathological process as well ; but so little at- 
tention has been paid to this difference in the autopsy reports, 
which have been published, that it seems an almost hopeless 
endeavor to distinguish whether the symptoms reported in a 
given case were due to changes in the cellular elements of the 
cortex itself, or to changes in the fibres of the medullary layer 
situated immediately beneath. Pick (Zeitsch. fur Heilkunde, 
1889, x, 1) has shown how important it is to make a micro- 
scopical examination ; this is even more necessary if we find 



CORTICAL LESIONS. 



I6 5 



secondary degenerations in the spinal cord — macroscopically 
the cortex may present no abnormality in such cases. 

We possess quite a considerable amount of material, but it 
is by no means easy to make a judicious and successful use of 
it. Certain methods have to be employed in order to arrive at 
correct conclusions, methods which have been developed in 
such an excellent way by Exner (cf. lit.). It would, for exam- 
ple, be incorrect to assume a certain cortical area to be the 
centre for the motor function of an extremity simply because 
in many cases a lesion of this area was found where a paralysis 
in that extremity had existed during life. This " method of 
positive cases," as Exner has called it, is therefore uncertain, 
because there are quite different circumscribed cortical regions, 
a lesion in which gives rise to the same symptoms ; and since, 
moreover, facts go to show that such a method may lead us to 
wrong conclusions, it ought to be discarded. Much more pref- 
erable, however, is the so-called " method of negative cases " 
(Exner), according to which " we have to mark out the lesions, 
found in all the cases in which a given function was not inter- 
fered with, and unite them on one hemisphere." If the number 
of cases is sufficiently large, while on the remaining parts of the 
cortex we find markings indicating lesions, the area for the 
functions in question will remain free. 

Still better results are obtained by the method of percent- 
ages (Exner). The cortex is divided into arbitrary fields, and 
for each of these fields we determine, first, how often it has 
been diseased in a given number of cases ; secondly, in how 
many of the cases the symptom which we are studying was 
present. The ratio between these two results is best given in 
percentages. Only through this indispensable, although some- 
what tedious, method can we ascertain that the fields of the 
right cortex are different from those of the left, and that cer- 
tain areas exist of which a lesion always, and others of which a 
lesion not always but frequently, produces a certain symptom. 
The former Exner calls " absolute," the latter " relative," corti- 
cal areas. 

We do not always find cortical lesions at the autops}^ in 
cases in which certain symptoms, which would have led us to 
suspect their existence, have been noted in life. On the other 
hand, they are found in cases where we have hardly felt justi- 
fied in expecting them. There is no doubt that no inconsider- 
able part of the cortex can be diseased without giving rise to 



1 66 DISEASES OF THE BRAIN PROPER. 




Fig. 35.— Right Hemisphere. (After Exner.) The portions shaded in represent those 
parts of the cortex which can be injured without giving rise to sensory or motor disturb- 
ances ; the blank areas are motor and sensory. 




Fig. 36. — Left Hemisphere. (After Exner.) This diagram shows that the motor and 
sensory areas are of greater extent on the left than on the right hemisphere. 



THE BRAIN CORTEX. 



167 



any symptoms. It is this part which has been called the cor- 
tical area of latent lesions (Exner), and it is certainly a matter 
worthy of note that the extent of this area is smaller on the 
left than on the right hemisphere, whereas the motor area — 
that is, the area in which a lesion is followed by motor disturb- 
ances — is larger and more developed on the left than on the 
right side (cf. Figs. 35 and 36). The first represents the right, 
the second the left hemisphere. On both all the lesions are 
indicated which have produced neither sensory nor motor 
disturbances. The blank fields are therefore sensory and 
motor — their greater extent on the left side is at once ap- 
parent. 




Fig. 37. — Convolutions and Fissures of the Lateral Aspect of the Brain. 
(After Ecker.) Parallelfurche = parallel fissure, or first temporal fissure. 

Before we go into the description of the individual lesions 
of the cortex we will briefly refresh our memory on the anat- 
omy of the parts. 

The thin gray covering which surrounds the white matter, and 
which has been called brain cortex, presents on each hemisphere 
three surfaces — a lateral, a basal, and a median. The two lateral 
form the convexity, the two basal the base of the cerebrum. The 
cerebrum is divided into lobes, which can again be subdivided into 
convolutions or gyri. To be able to localize and correctly describe 



i68 



DISEASES OF THE BRAIN PROPER. 



cortical lesions we must be thoroughly familiar with the position, as 
well as the names, of the different convolutions. The following 
illustrations are intended to facilitate the study of the convolutions 
and the fissures or sulci separating them. Fig. 37 represents those 
on the lateral surface (convexity), Fig. 38 those on the basal, and 
Fig. 40 those on the median aspect of the cerebrum. 




Fig. 38. — Convolutions and Fissures at the Base of the Brain. (Diagramatically 
after Ecker.) Hirnschenkel = crura cerebri. Balken = corpus callosum. 



In Fig. 37 are included the frontal, parietal, temporal, and oc- 
cipital lobes, so far as their convolutions and fissures belong to the 
lateral surface — in other words, belonging to the frontal lobes, the 
three frontal and the anterior central convolution (gyrus centralis 
anterior, pli frontal ascendant}, and belonging to the parietal lobe, 
the posterior central convolution (gyrus centralis posterior, or pli 
parietal ascendant) ; between the last two is seen the fissure of Ro- 
lando. Further, a part of the upper and the entire lower parietal 
lobe are shown, which latter is subdivided into the supra-marginal 



THE BR A IX CORTEX. 



169 



convolution in front and the angular gyrus {pit courbe) behind ; be- 
longing to the temporal lobe we have the three temporal convo- 
lutions, of which the first (uppermost) lies between the fissure of 
Sylvius and a very deep fissure running parallel to it, the so-called 
parallel fissure or first temporal fissure. The fissure of Sylvius has 
two branches, and the portion of the cortex between them is called 
the "operculum." Belonging to the occipital lobe, finally, there 
are three irregular and not always easily distinguishable occipital 
convolutions, between which two occipital fissures have been de- 
scribed. 

In order to determine from the outside of the skull the position 
of the fissure of Rolando we proceed, according to Kohler (Deutsch. 
Zeitsch. f. Chir., 1891, xxxii, 5, 6), in the following manner (cf. Fig. 
39) : A line, a, is drawn over the mid- a 

die of the skull from the forehead to 
the external occipital protuberance. 
A second line, b, is drawn at right 
angles with this, passing through the 
anterior boundary of the external 
auditory meatus ; parallel with this 
second line we draw a third line, c, 
passing through the posterior margin 
of the mastoid process, so that it cuts 
the sagittal line, a, two inches behind Fi 

the line c. A fourth line, d, starting 
from the point where a and c intersect, and running obliquely down- 
ward so that it meets the line b two inches above the external audi- 
tory meatus, will indicate the direction of the central fissure. 

On the basal aspect we see those parts of the three frontal con- 
volutions which are included in the base, of which the first (upper- 
most) is here called the gyrus rectus ; then the tractus, with the 
sulcus olfactorius ; next the uncinate gyrus, which belongs to the 
gyrus fornicatus, and which will be better seen on the median aspect ; 
further, the basal part of the third temporal convolution (gyrus tem- 
poralis inferior) and two lobules, which belong to both the temporal 
and occipital lobe, the inner (median) one called the lingual lobule, 
the one more external the fusiform lobule. 

The median surface (Fig. 40) shows in the middle the corpus 
callosum (in front the genu, behind the splenium) ; immediately be- 
low is the " septum lucidum," immediately above the gyrus forni- 
catus, the temporal part of which is called the hippocampal convolu- 
tion, and is continuous with the uncinate gyrus. Above.the gyrus 
fornicatus, and separated from it by the calloso-marginal fissure, are 
the frontal convolutions; farther back, the paracentral lobule, which 




170 



DISEASES OF THE BRAIN PROPER. 



meets the central convolutions. Behind this and belonging to the 
parietal lobe is the precuneus, and still farther back the cuneus (of 




Gyrus dentatus 

Tinibria or Tornix,. 

Fig. 40.— Convolutions and Fissures of the Median Aspect of the Brain. 
The posterior portions of the thalamus and the crus cerebri are cut off. 

the occipital lobe). The latter is bounded by two deep fissures — 
below by the calcarine, in front by the occipito-parietal fissure. 




Fig. 4 i. —Convolutions of the Island of Reil (J. R.) made visible by removing 

the Operculum. 



THE MOTOR CENTRES. 



171 



Extending from the posterior part of the corpus callosum to the 
uncinate gyrus is the hippocampal fissure. 

Covered by the above-mentioned operculum, in the depth of the 
fissure of Sylvius, is the lobus intermedins s. opertus, the so-called 
island of Reil, on which five to seven small convolutions are seen. 
Their position is shown in Fig. 41, where the operculum has been 
removed. 

In Fig. 42 the topographical relations between the surface of the 
brain and the surface of the skull are illustrated. 




Fig. 42. — Topographical Relations between the Exterior of the Skull and the 
Surface of the Brain. (After Ecker.) C, fissure of Rolando. V. C, anterior cen- 
tral, H. C, posterior central convolution. S6", -S,, fissure of Sylvius. T, temporal lobe. 
F, frontal lobe. P, , upper, P ti , lower parietal lobe. O, occipital lobe. Cd, cerebellum. 



As stated above, the localization of the motor centres by 
Fritsch-Hitzig and that of the speech centre by Broca paved 
the way for a number of discoveries which, based partly upon 
clinical observations, partly upon the less trustworthy experi- 
ments on animals, eventually will lead to a complete and ac- 
curate physio-pathological topography of the brain cortex. 
Thus far our knowledge is scanty and uncertain, and the centres 
which we shall here describe as being determined are almost 
all relative, in the sense of Exner (cf. page 165), the only excep- 
tion being the so-called motor region of the cortex. On the 
right hemisphere, the paracentral lobule, the anterior central, 



172 



DISEASES OF THE BRAIN PROPER. 



and the upper half of the posterior central convolution, on the 
left hemisphere, the paracentral lobule, the upper three fourths 
of both central convolutions, and a part of the upper parietal 
lobule, constitute the absolute cortical area for the upper ex- 
tremities (Exner). The absolute cortical area for the lower 
extremities is situated, on the right hemisphere, in the para- 
central lobule, and in the upper third of both central convolu- 
tions ; on the left hemisphere, in the paracentral lobule, the 
upper half of the posterior central convolution, and the greater 
part of the superior parietal lobule. 

The cortical area for the facial nerve is situated in the lower 
end of the anterior central convolution ; in front of this and in 
the adjoining portion of the second and third frontal convolu- 
tions is the centre for mastication (Hirt). In the region of the 
island of Reil we find the voice-centre — i. e., the centre for the 
movement of the vocal cords (Rossbach) ; in the frontal lobe 
that of the muscles of the neck (Fraenkel) ; in the angular 
gyrus that for the external ocular muscles. Haab (Zurich, 
1891) has attempted to determine the centre for the pupillary 
reflex. 

Of the so-called sensory centres — i. e., the areas in the cortex 
where conscious sensation takes place — we know the psycho- 
optic to be situated in the occipital lobe ; the psycho-acoustic 
in the temporal lobe ; that for smell and taste in the uncinate 
gyrus (Ferrier). The cortex of the frontal lobe and that of the 
temporo-occipital region are the seat of the higher intellectual 
processes (Flechsig). 

The so-called thermic centre discovered by Eulenburg and 
Landois corresponds to the motor region, and the tactile regions 
for the different parts of the body also are identical with the 
motor centres (Exner, Tripier). 

Further investigations must show whether the centres which 
we have been wont to regard as being situated in the medulla 
oblongata — for example, the centre for salivation, that for deg- 
lutition, that for the movements of the stomach and intestines 
(vomiting and defecation), for sneezing, coughing, etc. — are also 
situated in the cortex. The results of the treatment by sug- 
gestion make the assumption of such centres necessary. Nev- 
ertheless, while the " area of latent lesions " (Exner) is still as 
large as it is at present, an explanation of this kind is pre- 
mature. 



THE BRAIN CORTEX, 



LITERATURE. 



173 



A. Physiology. 

Hitzig. Ueber den heutigen Stand der Frage von der Localisation im Grosshirn. 
v. Volkmann's Samml. klin. Vortr., Leipzig, 1877, 113. 

Ferrier. The Functions of the Brain. G. P. Putnam's Sons, New York, 1886. 

Nothnagel. Topische Diagnostik, loc. cit., pp. 379 et seq. (many references). 

Munk. Zur Physiologie der Grosshirnrinde. Gesammelte Mittheilungen aus 
den Jahren 1877-1880. Berlin, 1881. Centralbl. f. Nervenhk., 1881, 17 ; 
1883, 12. 

Exner. Untersuchungen iiber die Localisation der Functionen in der Gross- 
hirnrinde des Menschen. Wien, 1881. 

Goltz. Ueber die Verrichtungen des Grosshirns. Bonn, 1881. 

Flechsig. Zur Anatomie und Entwickelungsgeschichte der Leitungsbahnen im 
Grosshirn des Menschen. Arch. f. Anat. u. Physiol., 1881, i, p. 12. 

Flechsig. Plan des menschlichen Gehirns. Leipzig, 1883. 

Goltz. Ueber die Verrichtungen des Grosshirns. 5 Abhandlungen. Arch. d. 
ges. Physiol., 1884, xxxiv, 9, 10. 

v. Gudden. Ueber die Frage der Localisation der Functionen der Gross- 
hirnrinde. Centralbl. f. Nervenhk., 1885, viii, 19. 

Christiani. Zur Physiologie des Grosshirns. Berlin, 1885. 

Goltz. Centralbl. f. Nervenhk., 1885, viii, 20, 21. 

Franck et Pitres. Recherches experimentales et critiques sur l'excitabilite des 
hemispheres cerebraux. Arch, de Physiol., 1885, 1, 2. 

Eulenburg. Ueber das Warmecentrum im Grosshirn. Verhandl. d. physiol. 
Gesellsch. in Berlin, 1885. 16. 

Luciani and Sepilli. Die Localisation auf der Grosshirnrinde (German by O. 
Frankel). Leipzig, 1886. 

Rossbach. Beitrag zur Localisation des corticalen Stimmcentrums beim Men- 
schen. Deutsches Arch. f. klin. Med., 1891, xlvi. 

Bechterew und Mislawski. Arch. f. Anat. u. Physiol., 1891, 5, 6. (Brain Cen- 
tres for Movements of the Vagina in Animals.) 

Ferrier. The Croonian Lectures on Cerebral Localisation. London, Smith, 
Elder & Co., 1890. 

Hosel. Die Centralwindungen, ein Centralorgan der Hinterstrange und des 
Trigeminus. Arch. f. Psych., 1892, xxiv, 2. 

Goldstein. Physiologie, Pathologie und Chirurgie des Grosshirns. Schmidt's 
Jahrb., 1892, ccxxxiii, p. 87. 

B. Pathology. 

Charcot et Pitres. Etude critique et clinique de la doctrine des localisations 

motrices dans l'ecorce des hemispheres cerebraux de l'homme. Paris, 

1883. 
Vetter. Ueber die sensoriellen Functionen des Grosshirns. Deutsches Arch. f. 

klin. Med., 1883, xxxii, p, 486. 
Alexander. Ein Fall von gummosen Geschwulsten in der Hirnrinde. Breslauer 

arztl. Zeitschr., 1884, 22. 
Rosenthal. Fall von corticaler Hemiplegie mit Worttaubheit. Centralbl. f. 

Nervenhk., 1884, vii, 1. 



1^4 DISEASES OF THE BRAIN PROPER. 

Berger. Zur Localisation cler corticalen Sehsphare beim Menschen. Breslauer 

arztl. Zeitschr., 1885, 1, 3-5. 
Desnos. Localisations cerebrales. Gaz. hebdom., 1885, xxxii, 47. 
Rumpf. Ueber syphilitische Mono-und Hemiplegien. Tagebl. d. Naturforscher- 

Vers. in Strassburg, 1885. 
Kohler. Zur Casuistik der reinen traumatischen Rindenlasionen. Charite- 

Annalen, 1886, xi, p. 538. 
Janeway. Journ. of Nerv. and Ment. Diseases, 1886, xii, 45. 
Bastian. The Muscular Sense, its Nature and Cortical Localisation. London, 

Clowes & Sons, 1886. 
Gowers. a. a. o., 1886, pp. 205 et seq. 
Reichard. Zur Frage der Hirnlocalisation mit besonderer Beriicksichtigung der 

corticalen Sehstbrungen. Arch. f. Psych, u. Nervenkh., 1886, xviii, 3 ; 

1887, xix, 1. 
Joffroy. Arch, de Physiol., February 15, 1887. (Monoplegia of the Lower Ex- 
tremities, Lesion in the Paracentral Lobule.) 
Leyden. Beitrag zur Lehre von der Localisation im Gehirn. Deutsche med. 

Wochenschr., 1887, 47. 
Bouveret. Lyon med., 1887, lvi, p. 337. 

Bernhardt. Ein Fall von Hirnrindenataxie. Ibid., 1887, 52. 
Horsley, Victor. Brain Surgery. Brit. Med. Journ., 1886, ii, 670-675. 
Ceci (Genova). Emiparesi progressiva sinistra iniziatasi due mesi dopo di un 

trauma alia regione parietale destra e giunta ad emiplegia completa. Rivista 

clin., Sett., 1887. 
Hun, H. A Clinical Study of Cerebral Localization. Amer. Journ. Med. Sc, 

1887. 
Nothnagel. Ueber Diagnostik bei Gehirnkrankheiten. Deutsche med. Wo- 
chenschr., 1887, xiii, 15, 16. 
Senger. Ueber topische Hirndiagnostik und Hirnchirurgie, etc. Deutsche med. 

Wochenschr., 1887, xiii, 10-13. 
Chauffard. De la cecite subite par lesions combinees des deux lobes occipitales. 

Revue de med., 1888, 2. 
Welt, Leonore. Ueber Charakterveranderungen des Menschen in Folge von 

Lasionen des Stirnhirns. Deutsches Arch. f. klin. Med., xiii, Heft 4. 

(Weakening of Intelligence, Deterioration of Character.) 
Dana. The Cortical Localization of Cutaneous Sensations. Journ. Nerv. and 

Ment. Diseases, 1888, xv, 650-684. 
Eckhardt. Neurol. Centralbl., 1889, 3. (Cortical Area for Secretion of 

Saliva.) 
Tomaczewski. Petersburger med. Wochenschr., 1889, 4. (Cortical Areas for 

the Nerves of Special Sense.) 
Lbwenthal. Deutsche med. Wochenschr., 1889, 15. 
Haab. Der Hirnrindenreflex der Pupille. Zurich, Miller, 1891. 

The speech centre, which, as we have seen, is situated part- 
ly in the frontal, partly in the temporal lobe of the left hemi- 
sphere, is certainly of larger extent than is commonly sup- 
posed. It is well known that after Bouillaud in 1825 had 
pronounced the frontal brain, and Marc Dax in 1836 the left 



APHASIA. 



175 



hemisphere, to be the seat of speech, Broca claimed that the 
posterior part of the third left frontal convolution, the pars oper- 
culars, or, as it later was called, the region of Broca, contained 
the speech centre ; and, indeed, in speech disturbances a lesion 
of this very region has most frequently been found at the au- 
topsy. There are, however, other parts of the cortex, as the 
island of Reil, the central convolutions, and, above all, the tem- 
poral lobe, more especially its upper convolution, which are 
connected with speech and which are of no less importance. 
Thus we have, after much laborious work and after many care- 
ful observations and comparisons, come to the conclusion that 
a different form of speech disturbance (aphasia) is produced 
according as the lesion is one of the frontal or of the temporal 
lobe — (of the left side only). In the former case the patient 
knows the word which he wishes to pronounce, but can not do 
so because he has lost the memory for the movements neces- 
sary for speech — i. e., he no longer knows how to use his tongue 
and lips in the act of speaking — motor aphasia. If the lesion is 
situated in the third left frontal convolution (Broca's region), we 
speak of cortical motor aphasia ; if it is situated in the white mat- 
ter of the hemispheres, in the posterior portion of the internal 
capsule, or in the left cms, we speak of subcortical motor aphasia. 

In the latter case — i. e., if the lesion is in the temporal lobe — 
the patient knows exactly what he wants to say, and he has no 
difficulty in repeating it 
if it is spoken for him ; 
but he can not find the 
expression for himself, 
he has "forgotten" the 
word — sensory aphasia. 
That the understandins: 
of words is situated in the 
temporal lobe, more par- 
ticularly in the first tem- 
poral convolution, was 
first stated by Wernicke, 
who also originated the 
terms motor and sensorv, 

cortical and subcortical, aphasia. The anatomy of the subcor- 
tical sensory aphasia has as yet been only imperfectly studied. 

In the diagram of Wernicke which is shown in Fig. 43, y 
represents the motor, x the sensory speech center ; the latter 




Fig. 43- 



Wernicke's Schema for the Cortical 
Mechanism of Speech. 



iy6 DISEASES OF THE BRAIN PROPER. 

is the terminus of the centripetal path of the auditory nerve 
a x, the former is the beginning of the centrifugal path y m 
going to the muscles used in speaking ; x y an assumed associa- 
tion path between both ; y is situated in the third frontal, x in 
the first temporal convolution. According as one or the other 
of these centres or the connection between them, or both cen- 
tres, were destroyed, Wernicke distinguished four cardinal 
types of aphasia: 

i. Destruction of the centre y — motor aphasia. Mobility 
of the muscles used in speech is retained, but the patient can 
either not speak at all or only say a few words or syllables. 
Understanding and memory of words are intact. 

2. Destruction of the centre x — sensory aphasia, " word 
deafness " (Kussmaul). The patient can use as many words as 
ever, but in speaking they are mixed up. The understanding 
of words is lost, although the power of hearing is not interfered 
with. 

3. Destruction of the association path x y, situated in the 
insula (?) — the so-called conduction aphasia of Wernicke. The 
patient can use as many words as ever, but in speaking they 
are mixed up. The understanding of words is retained. 

4. Destruction of both centres, x and y — total aphasia. 
Power and understanding of speech are lost. 

If we then consider as proved that a certain group of motor 
and sensory memory pictures are localized in the brain ; if we 
further agree that the former correspond to certain groups of 
muscles which serve a common purpose, the latter to the dis- 
tribution of a sensory nerve — it is not difficult to conceive that 
this same arrangement may exist for all the muscles and for 
all the sensory nerves. It is certainly easy to understand the 
occurrence of other motor defects in cases of aphasia. Thus 
there may be loss of simple movements (e. g., of the power to 
put out the tongue), or more complex ones (e. g., writing may 
become impossible — agraphia ; apliasie de la main, Charcot). 
Again, we have a patient who, in consequence of a cortical 
lesion in the central termination of the optic nerve, no longer 
recognizes his letters, and has thus lost the faculty of reading 
(" alexia ") ; or the visual memories may be lost altogether (not 
only those of letters), and a condition ensue which Munk calls 
psychical blindness. 

In examining a patient affected with aphasia, with a view 
of determining which path has become interfered with, we may 



APHASIA. iyy 

meet with considerable difficulty, and the diagnosis of the par- 
ticular type of aphasia with which we are dealing is often not 
easy, for the cases are not so sharply defined or so well charac- 
terized as we might be led to expect from the simplicity of 
the schemata. On the contrary, we often meet with combina- 
tions of the different types or with transition forms of aphasia 
in which even the most experienced clinician will venture a 
differential diagnosis only with much reservation. Take, for 
instance, the different degrees of that form of speech disturb- 
ance known as ataxic aphasia, in which the patient is unable to 
pronounce a word, though it constantly is floating, as it were, 
before his mind. This inability may go so far that the patient 
can only pronounce a few words or syllables (monophasia), 
that he involuntarily confounds words without being in the least 
uncertain about their meaning ; or it may, on the other hand, 
only amount to a slight disturbance, shown by some misplace- 
ment or omission of some letters, as in saving dy instead of 
dry, tnrk instead of truck, and the like. In the latter case we 
speak of syllable-stumbling (Silbenstolpern). Likewise we have 
different degrees of the so-called amnesic aphasia, where there 
may be loss or only slight impairment in the memory for words 
(sometimes only for words of foreign languages which have 
been learned later in life). As the faculty of writing and read- 
ing may often be more or less altered, it is important that it 
should be minutelv examined into ; the patient is asked to spell 
individual words, then to read sentences without spelling, then 
to write spontaneously and to dictation, and finally to copy 
words. In the case of a patient who is left-handed, his ability 
to write with the left hand should always be tested. Every 
case of aphasia must be carefully studied by itself, and each 
one gives opportunity for interesting observations. 

In general we may be guided by the following rules : 
i. If we find a patient whose sanity is established, who pos- 
sesses a normal acuteness of hearing and understands what is 
said, but is unable to repeat sentences or to speak spontaneous- 
ly, and can only utter individual words and syllables, we may 
assume a lesion of the third frontal convolution, possibly of the 
lowest part of the anterior central convolution. 

2. If a patient, although able to speak without difficulty, 
does not understand simple questions, then the first temporal 
convolution is diseased (in totd). If the understanding of words 
is only impaired, then only a part is affected. 



178 DISEASES OF THE BRAIN PROPER. 

3. If the patient has lost the faculty of reading, although 
there is no motor aphasia to be noted, we have to deal with a 
lesion of the cortical centre for vision (cf. page 172). 

4. A disease of the cortical speech centre does not exist if 
the patient gradually regains speech which he had suddenly 
lost ; if in such a case the hemiplegia, which has simultaneously 
appeared after an apoplectic stroke, persists, the white sub- 
stance near the cortex is usually diseased (Gowers). 

We should be going beyond the limits of this work if we 
attempted to discuss the aphasic symptom-complex in all its 
difficult and not rarely obscure details ; there exist a large 
number of interesting special articles on this subject, to the 
most important of which references will be found at the end of 
this chapter. While recognizing the steady advance which has 
been made toward the interpretation of these most complicated 
disturbances, we are ever reminded, by the constant difficulties 
which arise, how far we are from a complete understanding of 
them. Almost every case shows peculiarities which do not fit 
into any of the schemata ; and while to-day a successful investi- 
gator claims to have cleared up some obscure point in the diffi- 
cult field of aphasia, to-morrow another one proves that this 
conclusion was after all too hasty, and that only he, the sec- 
ond investigator, has really settled the question. In a word, 
there is hardly a single point in the problem of aphasia which 
is not still the subject of controversy. The tendency to schema- 
tize is very prevalent in Germany, and in opposition to these 
too schematic conceptions of aphasia, English and French in- 
vestigators have pointed out the variations of the inner speech 
— i. e., of the thinking processes necessary for speech — and 
the differences which may be bound up with the individual 
peculiarities of the person who speaks, writes, or reads. But 
these objections are slow to be appreciated in Germany. 
Whether a person reads by spelling, or whether after consid- 
erable practice one may read without spelling, whether the 
optical images of letters are necessary in the process of writing 
or not— these and many similar problems still await their solu- 
tion, and can be cleared up only by untiring, careful observa- 
tion of cases. 

For the beginner it is not only desirable but necessary to 
have the matter presented to him somewhat dogmatically, 
and this, according to our experience, will be best and most 
easily accomplished with the aid of schemata, of which, besides 



APHASIA. 



179 




path 

sions, 

path, 



a 



Fig. 44. 



the above-mentioned one of Wernicke, quite a number have 
been brought out. The one we have deemed most suitable and 
the best fitted for teaching 
purposes is probably that 
which Lichtheim has de- 
veloped (Arch. f. Psych., 
1884, xv, 3). It has been 
here given in Figs. 44, 45. 

The reflex arc neces- 
sary for repeating words 
contains the centre for au- 
ditory images of words, A ; 
the centre for motor im- 
ages, M; the centripetal 
for auditory impres- 
a A ; the connecting 
A M\ the centrifu- 
gal motor path, Mm. 
B is the place where 
concepts are formed — 
voluntary speech ne- 
cessitates a centrifugal 
path from B (brain cor- 
tex) to M. O is the 
centre for the visual 
images of letters. E 
is the centre for the in- 
nervation of the mus- 
cles required in writ- 
ing. Now, according 
to the path affected, we 
distinguish seven dif- 
ferent formsof aphasia. 

1. Interruption in 
the point M. Broca's 
(motor) aphasia. 

2. Interruption in 
point A. Wernicke's 
(sensory) aphasia. 

3. Interruption in the path 
(Wernicke). 

4. Interruption in the path MB 




! 



m 



i 



a 



Fig. 45- 

Figs. 44, 45.— Lichtheim's Schema illustrating the 
Seven Different Forms of Aphasia, a, A, cen- 
tripetal path for auditory impressions. A, centre for 
auditory images. M, centre for motor images. M, 
m, centrifugal motor path. B, the place where con- 
cepts are formed. O, the centre for visual images. 
£, centre from which the organs of writing are inner- 
vated. ( The seven different forms of aphasia have 
been indicated in Fig. 44 by the numbers 1-7. ) 



MA. Conduction aphasia 
A variety of motor apha- 



l8o DISEASES OE THE BRAIN PROPER. 

sia ; the faculty of repeating words and sentences being re- 
tained. 

5. Interruption in the path Mm. Variety of motor apha- 
sia ; the power of expressing thoughts in writing being re- 
tained. 

6. Interruption in the path A B. A variety of sensory apha- 
sia ; the patient being, however, able to repeat spoken language, 
to read aloud, and write from dictation. 

7. Interruption in the path A a. Inability to understand 
spoken language and to write from dictation, or to repeat spo- 
ken language. Nos. 6 and 7 have so far been observed only in 
rare instances (e. g., by Pick, Neurol. Centralbl., 1890, 21). 

As to the occurrence of aphasia, it is most frequently seen 
as a sequence to an apoplectic attack, either as a direct or indi- 
rect symptom ; in the latter case it is transient, and lasts, as we 
shall see later, a few minutes, hours, or days. In the former it 
persists, and may trouble the patient, though he may retain his 
full mental vigor to the end of his life. The most common 
form is motor aphasia, which appears in widely different grada- 
tions ; thus, in some cases the patient's speech may be just a 
little thick, while in others it may be altered so that it is no 
longer intelligible. After what has been said, it is easy to un- 
derstand that these defects chiefly occur after haemorrhage in 
the left side of the brain — that is, with a right-sided hemiplegia ; 
but it would be a decided error to suppose that they occur 
only or always in those cases, for motor aphasia may be found 
in connection with a left-sided hemiplegia, and it may be want- 
ing in the right-sided form. Other diseases of the brain also 
may implicate the cortical speech centre and give rise to 
aphasia. Among these may be mentioned general paralysis, 
psychoses (Lloyd, Francis, Lancet, July 7, 1888), processes of 
softening, chronic meningitis, tuberculous deposits, etc., and 
traumatism of the left hemisphere, in which case aphasia may 
be the only symptom. Aphasia has also been observed in acute, 
especially infectious, diseases — e. g., typhoid and scarlet fever. 
Most instances of this latter form occur in children. It has 
also been observed in the puerperal state. Of special interest is 
that form of total or motor aphasia which sometimes suddenly, 
sometimes gradually, comes on after a fright. That after a 
fright, such as makes " the hair stand on end," the voice may 
refuse to perform its duty, even Virgil seems to have known 
full well, as we see from the verse, " Steter unique coma, vox fan- 



APHASIA. j8i 

cibus hcesit." The nature of this form is uncertain, still it is by 
no means impossible that, just as we find that vaso-motor spasm 
acting on the facial vessels will produce pallor, so we may have 
a similar condition in those finest distributions of the middle 
cerebral artery which supply the region of Broca. That the 
spasm in these vessels is usually of longer duration and pro- 
duces more serious and more lasting consequences than the 
spasm of the cutaneous vessels, may be explained by the differ- 
ence in their arrangement, as well as by the difference in the 
function of the parts which they supply. 

It is not organic changes of the cortex which produce the 
symptoms in this case, the disturbances being entirely of a 
functional character, and this fright aphasia therefore consti- 
tutes a transition form to those instances in which, though the 
aphasia may have lasted for years, no changes are found at the 
autopsy, either in the cortical or subcortical area for speech. 
No doubt there is, besides the aphasia due to actual lesions in 
the cortex, also a functional form which we may imagine to 
originate in different ways, and it is at least probable that 
variations in the blood supply of the centres play an important 
part in this connection. Grashev (cf. lit.) has shown, in an in- 
genious piece of work, that we have to recognize a third form 
of aphasia, in which neither the centres nor the conducting 
paths are insufficient in their functions, but which is simply due 
to a diminished duration of the sensory impressions, giving rise 
to a disturbance in perception and association, and thus to an 
aphasic condition. Maybe it is this aphasia of Grashev which 
we find after concussion of the brain and after acute diseases, 
but it is difficult to diagnosticate it, and to differentiate a func- 
tional disturbance of the centres from a diminished duration 
of sensory impressions. A correct diagnosis is, however, of no 
small importance in the question of prognosis. . 

The outlook is absolutely unfavorable in cortical lesions 
where the centre is destroyed by processes of softening, tuber- 
culous deposits, atrophy of the gray cortex, etc., but is, of 
course, materially better if the centres remain intact, and are 
only temporarily rendered unable to perform their function, for 
then speech returns gradually, if not wholly, partially, and it 
can not be denied that systematic exercise and regular instruc- 
tion in speaking are capable of hastening an amelioration, nay, 
even a cure, especially if the patient be still young. 

The aphasia of children, which we sometimes find after 



1 82 DISEASES OF THE BRAIN PROPER. 

acute infectious diseases, fright, or as a consequence of intes- 
tinal worms ("reflex aphasia"), in the course of acute infantile 
cerebral palsy, or of epilepsy, and occasionally, but very rarely, 
after a cerebral haemorrhage, is in no other way to be distin- 
guished from the aphasia of adults except in its prognosis. Chil- 
dren, ccEteris paribus, always stand a better chance of improve- 
ment or recovery from aphasia than adults, no doubt because 
it is easier to educate in them the well half of the brain to per- 
form the function of the damaged one. If the disturbance is 
only functional, as I saw in one case which was due to an over- 
dose of santonin, in which the disorder in speech only lasted a 
few hours, the outlook is still more favorable, and complete 
recovery may be confidently expected ; but if the function of 
one speech tract — that is, the left — be impaired by cortical or 
deep-seated lesions, even then it is in children usually not very 
difficult to educate the right side to some vicarious action, espe- 
cially in cases where, before the lesion, the children have been 
taught to use both hands equally. The possibility of a cere- 
bral disease should be thought of in the gymnastic cultivation 
and development of the body of children ; the extremities of 
both sides should be exercised and strengthened equally, the 
children should be made ambidextrous ; only then can, in a 
case of necessity, the right hemisphere fully take the place of 
the left. 

A treatment for the aphasia as such does not exist. In 
cases of functional aphasia the only thing necessary is to con- 
vince the patient that his condition is not serious. If this does 
not lead to any improvement, we should try hypnotism, from 
which astonishing results have sometimes been obtained. If, 
on the other hand, the aphasia is due to organic changes, such 
as haemorrhage or embolism, in our treatment we must be 
guided by the principles discussed in the chapter dealing with 
these conditions. 

LITERATURE. 

Wernicke. Lehrbuch der Gehirnkrankheiten, 1881, pag. 366. 

Kussmaul. Die Storungen der Sprache. 3. AuflL, Leipzig, 1885. 

Charcot. De l'aphasie en general et de l'agraphie en particulier. Progres 

med., Fevr. 5, 1888. 
Caro. Deutsches Arch. f. klin. Med., 1888, xliii, 2, 3. 
Landolt. De la cecite verbale. Travail publie dans l'ouvrage dedie a Mr. 

Donders a l'occasion de son jubile. Utrecht, 1888. 
Malachowski. Versuch einer Darstellung unserer heutigen Kenntnisse in der 

Lehre von der Aphasie. Volkmann's Samml. klin. Vortr., 1888, No. 324. 



CORTICAL MOTOR PARALYSIS. ^3 

Adler. Beitrage zur Casuistik und Theorie der Aphasie. Dissert. Inaug., 

Breslau, 1889. 
Berkhan. Ueber Storungen der Sprache und der Schriftsprache. Fur Aerzte 

und Lehrer. Berlin, 1889. 
Eisenlohr. Deutsche med. Wochenschr., 1889, 36. 
Soltmann. Ueber Schrift und Spiegelschrift bei gesunden und kranken Kindern. 

Henoch-Festschrift, 1890. 
Ballet. Die "innerliche Sprache" und die verschiedenen Formen der Aphasie. 

2. Aufl. German by Bongers, 1890. 
Leube. Ueber einen eigenthumlichen Fall von Alexie. Zeitschr. f. klin. Med., 

1890, xviii, 1, 2. 
Mills, Charles K. Aphasia. Review of Insanity and Nervous Diseases. Sep- 
tember-October, 1 891. 
Bateman. On Aphasia or Loss of Speech, etc. London, 1891. 
Adler. Beitrag zur Kenntniss der seltenen Formen von sensorischer Aphasie. 

Neurol. Centralbl., 1891, 10, 11. 
Bianchi. Contributo clinico ed anatomo-patologico alia dottrina della cecita 

verbale. (Afasia ottica) Annal. de Nevrol., Nuova Ser., 1891. 
Freud. Zur Auffassung der Aphasien. 1891. 
Moeli. Berliner klin. Wochenschr., 1890, 17; and 1891, 48. 
Lowenfeld. Ueber zwei Falle von amnestischer Aphasie nebst Bemerkungen 

iiber die centralen Vorgange beim Lesen und Schreiben. Deutsche Zeitschr. 

f. Nervenhk., 1892. 
Goldscheider. Berliner klin. Wochenschr., 1891, 20; 1892, 4. 
Pick. Arch, f. Psych., 1892, xxiii, 3. 

Leichtenstern. Ueber die Schreibweise Linkshandiger. Deutsche med. Wo- 
chenschr., 1892, No. 42, pag. 942. 
Simon, J. Ueber amnestische Aphasie. Inaug.-Dissert., Berlin, 1892. 
Sachs, Heinrich. Vortrage iiber Bau und Thatigkeit des Grosshirns und der 

Lehre von der Aphasie und Seelenblindheit. Breslau, 1893. 
Gutzmann. Vorlesungen iiber die Storungen der Sprache und ihre Heilung. 

Berlin, 1893. 
Gossan. Zwei Falle von Aphasie. Arch. f. Psych., etc., 1893, xxv, 1. 

Knoblauch. Ueber Storungen der musikalischen Leistungstahigkeit in Folge 
von Hirnlasionen. Deutsches Arch. f. klin. Med., 1888, xliii, 4, 5. 

Kast. Ueber musikalische Storungen bei Aphasie. Neurol. Centralbl., 1888, 14. 

Oppenheim. Musikalische Ausdrucksbewegungen bei Aphasie, etc. Schmidt's 
Jahrb., 1891, ccxix, Heft 8. 

Kunn. Die Tontaubheit. Wiener med. Wochenschr., 1892, 9-13. 

Treitel. Aphasie im Kindesalter. Volkmann's Samml. klin. Vortr., 1893, 64. 

We have repeatedly pointed out that the motor disturb- 
ances produced by cerebral diseases are either due to destruc- 
tive or irritative lesions. The former consist of paralyses and 
pareses, the latter of involuntary movements in different groups 
of muscles — the so-called spasms. Those disturbances which 
are due to affections of the cortex (cortical motor disturbances) 



8 4 



DISEASES OF THE BRAIN PROPER. 



present much that is characteristic and interesting. They wilt 
be considered presently. 

The motor centres, the motor area of the cortex, comprise, 
as has been stated above, the two central convolutions, the 
paracentral lobule, and the parts lying immediately adjacent. 
Upper and lower extremities, neck and face, have their own 






/ / 

J / 

i / / 

<t% //^^^^^ 

^fX^—^-----"' 

Lenticular nucleus \ ~y$? .-'" •«<. — 

h— . — . . — Pyramidal tract. » \' V.. ^--sTp—^- Nucleus dentatus 

\ *A \" v • • \~*^S& cerebelli 

• • Frontal cortico-pontine • *\v •- ^^A"^ 

fasciculi. \^ LX^ 

.. Temporo-occipital corti- I " * ^ _ — ^f "' 

co-pontine fasciculi. V^^-s 

Ant. and post, cerebello- * *V 

pontine fasciculi. « • 

Tegmental radiation and » I 

sup. peduncle of cere- « \ 
bellum. % • 

Ansa lenticularis. 1 \ 

\ $ Crossed pyramidal tract 

x^— Xj — Xj — 2t — 21 Cortical fillet (psycho- 1 

sensory paths). ^ Uncrossed pyramidal tract 

I 
Fig. 46.— Diagram showing the Direct System of Fibres (Flechsig, Mendel). 

special centres, which are distinctly separated from one an- 
other in the central convolutions. Thence fibres converge, 
as is shown in Fig. 46, into the anterior two thirds of the 
posterior division of the internal capsule. One thing at once 
becomes apparent from this arrangement, namely, that in cor- 
tical lesions the paralysis or paresis may easily be confined 
to one extremity, an arm or a leg, while if the lesion affects 




CORTICAL MOTOR PARALYSIS. 



85 



the tract lower down in the brain — for instance, in the region 
of the capsule — the paralysis must extend over the whole half 
of the body. A paralysis of one extremity only is called a 
monoplegia (monoparesis), in contradistinction to a hemiple- 
gia, which means paralysis of one side (half) of the body, and it 
is a perfectly warrantable conclusion, sufficiently confirmed by 
post-mortem evidence, that, if the patient presents a paralysis 
of only one arm or one leg, we are dealing with a cortical lesion. 
A hemiplegia is only then likely to be of cortical origin if its- 
development indicates that the lesion beginning in one motor 
centre has gradually encroached upon another. It goes without 
saying that in diagnosticating cortical lesions we must not rely 
on a single symptom, but all must be considered, and especial 
care must be taken not to confound a paralysis of cortical with 
one of peripheral origin. One great distinction between these 
two is to be found in the manner of onset. While a paralysis 
of cortical origin may develop quickly in a few hours, a periph- 
eral one will be more gradual, and only reach its full extent 
after weeks or even months. Moreover, the latter, the periph- 
eral, is easily recognizable by the changes which take place in 
the electrical excitability — e. g., if reaction of degeneration and 
visible atrophy in the muscles can be demonstrated. The ab- 
sence of cerebral symptoms, which are rarely entirely wanting 
in cortical affections, is also characteristic of peripheral disease. 
Great pain may be entirely absent in the central, but is com- 
monly present to a greater or lesser degree in the peripheral 
variety. Remembering, then, these points, and making it a 
routine practice never to omit the electrical examination in 
doubtful cases, we are not likely to make an error in the 
diagnosis. 

In cortical lesions the loss of motion is usually not absolute, 
and we find more frequently a paresis than an actual paralysis. 
The disorder does not necessarily affect a whole extremity, an 
arm or a leg; it may be confined to the distribution of special 
nerves, or even to portions of these, the so-called dissociated 
hemiplegias (cf. also Pick, Prag. med. Wochenschr., 1891, 25-27). 
Sometimes the affected arm or leg can be moved in toto, though 
a strong effort may be required, and it is only in the fingers 
and toes that the loss of power is complete. 

A characteristic symptom is the inability of the patient to 
execute complicated movements, such as buttoning his coat, 
counting money, and so forth, acts which are performed awk- 



1 86 DISEASES OF THE BRAIN PROPER. 

wardly and with difficulty, owing to a loss of the motor im- 
ages. This condition has been called ataxia, and in these cases 
we have a " cortical ataxia." The lesion has to be referred to 
that part of the cortex which contains the sensory area {Filhl- 
sphdre of Munk) for the affected, that is, the ataxic extremity. 
The trouble is very distressing to a patient in a brachial as well 
as in a crural monoplegia, and becomes almost unbearable if the 
sensory disturbances, which we shall shortly describe, are super- 
added (cf. lit., Observations of Bernhardt). 

In infective tumors, gummata, tubercles at the surface of 
the brain, we occasionally meet with symptoms of irritation, 
such as monocontractures, which depend upon an irritation in 
the corresponding portion of the motor path (Wernicke). They 
are not seldom accompanied by sharp pains. In such cases the 
differential diagnosis between a hysterical and a true organic 
cortical lesion may cause considerable difficulty (cf. chapter on 
Hysteria). 

Of the greatest practical importance are the epileptiform 
attacks which occur, either with or without loss of conscious- 
ness, as a consequence of direct or indirect irritation of the 
cortex. If they occur in the further course of the monoplegia, 
the onset of which was apoplectiform, the diagnosis of a corti- 
cal lesion can be made with a high degree of probability. In 
some cases the convulsions are not general, but only appear as 
localized twitchings or spasms, confined to one half of the body 
or one extremity ; they may be clonic (that is, an alternation in 
quick succession of contractions and relaxations) or tonic (that 
is, steady contractions lasting for some time), and may be of 
considerable intensity ; their occurrence later in parts already 
paralyzed would indicate a disease of the brain surface, though 
we may not always be able to say whether the irritation of the 
cortex depends upon a direct or — as, for instance, in tumors, 
which cause an increase of the intracranial pressure — an indi- 
rect action. In the latter case, also, general or partial convul- 
sions may ensue. 

The use of the term cortical epilepsy (or Jacksonian epi- 
lepsy, after Hughlings Jackson, who first described these con- 
ditions) is liable to give rise to misconceptions, and it must be 
remembered that the so-called cortical epilepsy has nothing in 
common, except the name, with the classical genuine epilepsy. 

The epileptiform seizures due to cortical lesions show cer- 
tain fundamental differences from the classical attacks. Con- 



CORTICAL MOTOR PARALYSIS. 



I8 7 



sciousness is retained, a feature which gives the whole attack 
an entirely different aspect. A certain kind of aura occurs 
here also ; the patient knows when the convulsions are coming 
on, either by slight twitching in the fingers or toes, or by for- 
mication and other symptoms, which occur only in the affected 
extremity. But all the other symptoms — the cry, the fall, the 
biting of the tongue, etc. — are absent. The patient sees and 
watches the twitching of his extremity ; not rarely he has vio- 
lent pains ; he tries to hold the extremity in a fixed position or 
asks others to do so, and attempts to avoid injuring himself. 
After the convulsions he feels weak and unstrung, but only in 
consequence of the increased muscular work. Headache and 
all the various post-epileptic symptoms are absent, or, at any 
rate, are not connected with the attack as such. 

The degree, the duration, and the frequency of the attacks 
vary considerably; sometimes only a more or less marked 
twitching appears in the affected limb; sometimes, however, 
the attack manifests itself in shaking movements, which may 
become so violent that the bed shakes and the patient anxiously 
cries for some one to assist him and to hold him. If violent 
pains have been present during the movements, they are wont 
to persist after the attack, and, combined with the motor weak- 
ness in the affected extremity, are often productive of great 
suffering. The duration also varies. 1 have seen cases in 
which the attack was over in from a quarter of a minute to one 
minute ; on the other hand, I have seen instances in which it 
has lasted for a quarter of an hour. If such prolonged attacks 
occur at frequent intervals — two, three, or six times a day — the 
state of the patient may be very pitiable ; and, indeed, the car- 
rying on of the individual's occupation may be interfered with 
by this partial epilepsy much more than it often is in cases of the 
classical disease. In other instances months intervene between 
the attacks. The whole course of the malady is eminently 
chronic ; the patient may suffer for years, or tens of years, with- 
out there being any other symptom present. Death occurs 
either from an extension of the brain lesion or as a result of 
some intercurrent disease. Pitres has called attention to the 
fact that so-called equivalents may occur in Jacksonian epilepsy 
also, and has pointed out that they may be of a sensory or of a 
psychical nature (Revue de med., 1888, viii) ; the former belong 
to Charcot's epilepsie partielle sensitive (Lecons du Mardi a la 
Salpetriere, 1889, pp. 20 and 368) ; the latter manifest themselves 



1 88 DISEASES OE THE BRAIN PROPER. 

in visual, auditory, or olfactory hallucinations without any 
marked signs of motor irritation. 

With reference to the diagnosis, it should be mentioned 
that, just as in the case of genuine epilepsy, cortical epilepsy 
may be simulated by urasmic attacks if the latter are confined 
to one side (Chauffard, De l'uremie convulsive a forme de l'epi- 
lepsie Jacksonienne, Arch. gen. de med., July, 1897). Fur- 
thermore, attacks which resemble very closely those of Jack- 
sonian epilepsy may occur in hysteria ; in these cases the 
presence of other hysterical manifestations will prevent an 
error in diagnosis. Mendel has repeatedly observed cases of 
general paralysis in which Jacksonian epilepsy was the initial 
symptom. The foci which were found at the autopsy were in 
each case situated in the right psycho-motor region, and the 
(paralytic) speech disturbance occurred in the terminal stage, 
whereas usually this is one of the early symptoms of general 
paralysis. 

The sensory disturbances which are produced by the affec- 
tions of the brain cortex are remarkable, and by no means fully 
understood. As we have seen before, they do not, as a rule, 
cause pain, but rather manifest themselves in alterations of 
sensation, known as parassthesias. Thus the patient may speak 
of a curious numbness or deadness ; or, again, he may have a 
sensation as of ants crawling under the skin, a feeling as if the 
part had gone to sleep, etc. There may also be a distinct in- 
crease in pain perception, a slight " analgesia," a diminution or 
loss of pressure, touch, and temperature sense, and oftener, as 
it seems, in diseases of the parietal lobes a more or less pro- 
nounced disturbance of the muscular sense, in consequence of 
which the patient can with closed eyes either give no account 
at all or only a very imperfect one of the position of his ex- 
tremities. If, as often happens, the above-described awkward- 
ness in motion (ataxia) coexists with these changes, we may be 
tempted to refer the trouble not to the cortex, but to the spinal 
cord ; more especially are we liable to think of tabes, although 
the ataxia is produced in an entirely different manner in the 
two diseases. However, the differential diagnosis will in most 
cases present no difficulties if we take into consideration all the 
symptoms, and examine into the condition of the patellar re- 
flexes, the reaction of the pupils, and ascertain whether there 
are bladder symptoms and whether lancinating pains are pres- 
ent or not. These sensory changes, we must not forget, are 



THE CENTRUM OVALE. 



8 9 



by no means always observed in cortical lesions, and in the 
cases in which they existed the white matter of the brain has 
often been found to be likewise the seat of disease ; they are 
therefore in no way to be regarded as pathognomonic, and we 
have to be cautious in using them for diagnosis. The same is 
true to a greater degree of the vaso-motor and trophic changes, 
the relation of which to the brain cortex is still obscure. 

Symptoms Referable to Lesions of the White Matter of the 
HcmispJieres and Lesions of the Basal Ganglia. 

Looking at the anatomy of the parts, we notice that the fibres 
coming from the cortex pass through the white matter of the hemi- 
sphere, which in the region of the frontal and parietal lobes is 



Frontal lobe 



Anterior limb 
of internal 



2Vwc7. caudat 




Posterior limb 
of internal 



Thalamus. 



Cms cerebri 



Fig. 47. — Course of the Fibres from the Internal Capsule to the Crus Cerebri. 
The thalamus is represented as transparent. (Diagrammatic after Wernicke and 
Edinger.) 



designated centrum semiovale Vieussenii. Turning toward the brain- 
stem, in its neighborhood they appear arranged in bundles placed 
side by side, completing by their convergence what has long been 
known as the corona radiata. With this corona radiata begins the 



9 o 



DISEASES OF THE BRAIN PROPER. 



great nerve tract which connects the hemispheres with all parts of 
the brain situated lower down, and finally with the spinal cord. That 
part of the medullary path through which the corona radiata is con- 
tinued into the crura cerebri is called the internal capsule. As is 
seen in Fig. 47, this is situated anteriorly between the caudate and 
the lenticular nucleus, posteriorly between the lenticular nucleus 
and the optic thalamus. The point where the two segments meet is 
called the genu or knee of the capsule. 




Corp. cattos. 

Ventric. later, 
Vektric. sept, pelluc. 
—Nucl. caud. 

JFormx. 
— Commiss. anter. 

Commiss. media 
Taenia semicircularis 

Thalamus 

Commiss. poster. 

Pineal gland 

Xulvinar 

Corpora, 
' igemina 



Fig. 48.— View of the Ventricles on Horizontal Section. (After Edinger.) 



From the internal capsule the fibres reach the crusta (pes pedun- 
culi cerebri), whence they pass through the lower (anterior or ven- 
tral) portion of the pons and enter the medulla oblongata as the 
anterior pyramids. At the lower end of the medulla most of them 
decussate and pursue a downward course in the lateral columns of 
the spinal cord on the opposite side. This, the most important of 



THE INTERNAL CAPSULE. 



I 9 I 



all direct systems of fibres, was discovered by Deiters in 1865, and 
most carefully studied by Flechsig in 1876. It is generally known 
as the lateral pyramidal tract, and it represents the path for the 
voluntary movements. A lesion of it is therefore of grave conse- 
quence for the motor functions. 

Fig. 48 represents a horizontal section which shows the relative 
position of the caudate nucleus to the optic thalamus, the corpus 
callosum, the fornix, the two white commissures, the anterior and 
the posterior, the gray middle commissure, the pineal gland, and the 
corpora quadrigemina. 

Fig. 49 is a third horizontal section through the cerebrum at a 
lower plane. Both are taken from Edinger. 



_-*- Tor nix 

IS 




Fig. 49. — Horizontal Section through the Brain, about a Finger's Breadth 

BELOW THAT REPRESENTED IN FlG. 48. (EDINGER.) 



Figs. 50, 51, and 52 are three so-called frontal sections, of which 
the first is made through the anterior commissure, the second in 
front of, the third behind, the middle (gray) commissure. They also 
show the course of the internal and external capsule, and the situa- 
tion of the so-called basal ganglia, the caudate and the lenticular nu- 
cleus (together known as the corpus striatum), and the optic thalamus. 



192 



DISEASES OF THE BRAIN PROPER. 



In another frontal section, Fig. 53 (after Edinger), the direction 
of the fibres is illustrated diagrammatical!}^. 




Corpus callosum 
Sept. jpettuc. 

JSticl. caudat. 

Cajps. int. 

Nucl. lentif. 

Claustrum 



Fig. 5°- 

Pitres has recommended a series of frontal sections in order to 
facilitate in our descriptions of autopsies a more accurate localiza- 



Miel. caud. 



Corpus callosum 
Crura forn. 
Thalam. 
Insula 
Caps. int. 
JKucT. hntiform, 
Caps. ext. 
Claustr. 




Fig. 5i. 

tion of lesions and tumors within the very extensive white matter 
of the brain. Nothnagel has modified somewhat these sections of 



PITRES' SECTIONS. 



193 



Pitres with regard to their position and designation. The table on 
page 194 contains the necessary explanation. With the help of these 
sections we need not content ourselves any longer with locating in the 



Micl caud. 

JFornix 
Thalamus 



Commissura mollis 
(media) 
Nucl. tegment.—Ur 
(red nucleus) ,,, ^^ 
Subst. nigr. 
Pes peduncuh 




CORO/VA 
/(AD/ATA 

Go/MGTO 
orr/c 



%J^< 



ofT/f/tuiMi/sX 

Fig- 53- 
Figs. 50-53. — So-called "Frontal Sections" through the Brain. Fig. 50, through 
the anterior commissure. Fig. 51, in front of the middle commissure. Fig. 52, behind 
the middle commissure. Fig. 53, immediately behind the chiasm. The radiating fibres 
are shown diagrammatically in the last illustration. (After Edinger.) 
13 



94 



DISEASES OF THE BRAIN PROPER. 



post-mortem accounts a tumor "in the anterior part of the brain," "in 
the temporal lobe," etc., but we give the one or more sections which 
correspond to the situation of the neoplasm, and so attain an accu- 
racy which is indispensable for the after-use of our autopsy records. 

PITRES-NOTHNAGEL FRONTAL SECTIONS. 



Designation. 


Points where sections are 
made. 


Called by Pitres 


Comprises, according to 
Nothnagel, 


A 


Immediately in front of 
genu of corpus callo- 
sum. 


Coupe prefrontals 


Centri ovalis pars frontalis 
anterior. 


B 


Starting at the beginning 
of fissure of Sylvius. 


1 r 

1 Coupe pediculo- 1 
1 frontale. 1 

I I 

J I 


Pars frontalis media. 


B, 


Between anterior cen- 
tral and frontal con- 
volutions. 


Pars frontalis posterior. 


C 


Through the fissure of 
Rolando. 


Coupe frontale. 


Pars centralis anterior. 


D 


Through ascending parie- 
tal convolution. 


Coupe parietale. 


Pars centralis posterior. 


E 


Through parietal lobe 3 
cm. posterior to the 
fissure of Rolando. 


Coupe pediculo-pa- 
rietale. 


Pars parietalis. 


F 


Through occipital lobe. 


Coupe occipitale. 


Pars occipitalis. 




Fig. 54. — Points at which the Pitres-Nothnagel Sections are made. They all 
run parallel with the fissure of Rolando. 

Figs. 55-60 represent diagrammatically Pitres' sections. From 
Fig. 54 we can get an idea of the points on the surface of the brain 



LESIONS OF CENTRUM OVALE. 



195 



at which the sections are to be made (cf. Pitres, Recherches sur les 
lesions da centre ovale des hemispheres cerebraux etudies au point de 
vue des localisations cerebrales, Paris, 1877). 

1 



Section A 





Fig'- 55- — !> 2 i 3) first, second, and third frontal convolutions. 4, prefrontal fasciculus of 

the centrum semiovale. 



We can likewise avail ourselves of the charts of the human brain 
published by Exner (two plates, with twelve diagrams, Wien, Brau- 
miiller, 1888). On the plates the discovered lesion can be easily 
marked off, and thus the extent and situation of it represented. 



Section B. (B x Nothnagel 





Fig. 56. — 1 and 2, first and second frontal convolutions. 3, middle pediculo-frontal fascicu- 
lus. 4, corpus callosum. 5, nucleus caudatus. 6, internal capsule. 7, lenticular nucleus. 
8, island of Reil. 9, 10, n, superior, middle, and inferior frontal fasciculi. 

With reference to the lesions in the centrum ovale, it should 
be stated that, as a rule, the symptoms produced by them are 
similar to those which we find in lesions of the corresponding 



196 



DISEASES OF THE BRAIN PROPER, 
Section C. 




Fig. 57. — 1, first frontal convolution. 2, 3, 4, superior, middle, and inferior frontal fasciculi. 
5, corpus callosum. 6, nucleus caudatus. 7, optic thalamus. 8, internal capsule. 9, len- 
ticular nucleus. 10, claustrum. 

area of the cortex. Thus we shall meet with motor disturb- 
ances if the fronto-parietal fasciculi of the corona radiata, which 



Section D. 




Fig. 58.— 1, anterior central convolution. 2, corpus callosum. 3, caudate nucleus. 4, optic 
thalamus. 5, internal capsule. 6, island of Reil. 7, lenticular nucleus. 8, external 
capsule. 9, temporal fasciculus. 10, n, 12, superior, middle, inferior parietal fasciculi. 



LESIONS OF CENTRUM OVALE. 



I 9 7 



take their origin in the motor area, are diseased ; while lesions 
in the prasfrontal or occipital bundles may, and indeed very 
often do, not evoke any symptoms. If the left (inferior) pedic- 



Section E. 




Fig. 59. — 1, superior parietal fasciculus. 2, corpus callosum. 3, inferior parietal fasciculus. 
4 and 6, caudate nucleus. 5, optic thalamus. 



ulo-frontal bundles in addition are affected, the patient will 
also be aphasic, the aphasia, however, being of long duration 
only if the lesion extends close up to the cortex. Lesions in 



Section F. 




Fig. 60. 
Figs. 55-60. 



-1, occipital convolutions. 2, occipital fasciculus of the centrum semiovale. 
-PiTRES-NoTHNAGEL Sections, the position of which is made clear by the 
table on page 194. 



the white matter of the occipital lobe may produce hemianopia, 
in the temporal lobe auditory disturbances. Whether, however, 
diseases in the parietal lobes ever produce sensory changes — 



I98 DISEASES OF THE BRAIN PROPER. 

anaesthesia, for instance — and whether, as a consequence of any 
lesion in the centrum ovale, vaso-motor-trophic changes may be 
developed, is unknown. 

The idea that the basal ganglia were true motor centres, and 
that the common form of hemiplegia was due to lesions thereof, 
has been given up, and we have learned from the investigations 
of Flechsig and Wernicke that direct connections between the 
motor centres of the cortex and these basal ganglia do not 
exist. Moreover, it has been proved by numerous thoroughly 
reliable observations that destruction of the lenticular or of the 
caudate nucleus does not necessarily give rise to a motor pa- 
ralysis. One or both lenticular nuclei have repeatedly been 
found destroyed in cases in which there was no sign of paraly- 
sis (Lepine, Nothnagel, Edinger, Heboid). In order that this 
may not ensue, it is only necessary that the internal and per- 
haps also the outer capsule remain intact. As soon as the for- 
mer (the inner capsule) is either directly or indirectly im- 
plicated, we have a hemiplegia which is either transient or 
persistent, according to the nature of the lesion in the capsule. 
Whether the lenticular or the caudate nucleus alone is diseased 
can not be determined from the symptoms. 

There is no doubt but that lesions of the thalamus, espe- 
cially of its anterior and middle part, may occur without 
symptoms, and it is impossible to say whether motor paralysis 
is ever produced by lesions of the thalamus, for in all instances 
in which this may have been the case the motor paralysis may 
also have been a result of damage to neighboring parts (pedun- 
cles, internal capsule). 

Better founded is the idea that lesions of the pulvinar, the 
posterior part of the thalamus, give rise to defects in sight — 
crossed amblyopia or homonymous bilateral hemianopia ; but 
the possibility that the posterior part of the optic tract is inter- 
rupted can even then not be excluded. The athetoid move- 
ments and symptoms of motor irritation (hemichorea, post- 
hemiplegic tremor, athetosis) are, even if a connection actually 
exists between them and lesions of the thalamus (Greif, cf. lit.), 
certainly not characteristic of such lesions. The same holds 
good for the disturbances in the muscular sense which have 
been observed in diseases of the thalamus (Meynert, Jackson). 
The relation between these latter and loss of the movements of 
facial expression in the course of central facial paralysis has 
been spoken of in Part II, Chapter V. Recently Nothnagel 



THE CORPORA QUADRIGEMINA. 199 

has again published a clear case of this kind (Zeitsch. f. klin. 
Med., 1889, xvi, 5, 6, p. 424). 

Lesions of the internal capsule produce symptoms varying 
according as the anterior or posterior limb is attacked. Pure 
capsule lesions — i.e., those in which the caudate as well as the 
lenticular nucleus remain intact — have rarely if ever occurred. 
Fissures have been occasionally known to occur without hav- 
ing necessarily produced any motor disturbances in life (cf. 
Nothnagel, loc. cit., p. 272). The functions of the anterior limb 
of the internal capsule are obscure, and lesions of this part do 
not produce any symptoms. With the posterior limb we are 
better acquainted, and, above all, this one fact is well estab- 
lished, that a lesion of the anterior two thirds of the posterior 
limb gives rise to the usual typical hemiplegia, with paralysis 
of the lower facial branches. A very small lesion at the knee 
may produce an isolated facial paralysis. If the posterior por- 
tion of the anterior two thirds is the chief seat of the disease, 
the paralysis is most marked in the leg. The posterior third 
of the posterior limb is occupied by the sensory fibres (le carre- 
four sensitif of Charcot), and lesions of that region cause a loss 
of sensation on the opposite side of the body (" hemianesthe- 
sia," Oppenheim, Charite-Annalen, 1889, xiv, p. 396), in which 
often the nerves of special sense are implicated, and hearing, 
smell, and taste (on the anaesthetic side) are, if not lost, at least 
diminished. Often hemiplegia is accompanied by hemianaes- 
thesia, because, if the one portion of the capsule is affected, an 
indirect and transitory implication of the other may occur. Usu- 
ally such a hemianaesthesia soon disappears in the same way as 
the indirect motor disturbance often soon passes off in cases of 
persistent hemianaesthesia. Whether the symptoms of motor 
irritation (the so-called post-hemiplegic chorea, for instance), 
which are a not rare accompaniment of hemiplegia, are due to 
disease of the internal capsule or to disease of the neighboring 
basal ganglia, is as yet undecided. 

By the corpora quadrigemina we mean that peculiar eminence 
which by a crucial furrow is separated into four parts (bodies), and 
forms the posterior boundary of the third ventricle. In front it is 
bounded by the commissure which unites the two thalami ; on it 
rests the pineal gland (conarium). The anterior pair of bodies, 
which are called the nates, are larger than the posterior, the testes. 
The appearance and structure which these two pairs of bodies pre- 
sent in the lower mammals, justifies the conclusion that they are to- 



200 



DISEASES OF THE BRAIN PROPER. 



tally different from each other. Above the corpora quadrigemina is 
situated the spleniumof the corpus callosum ; between the two is the 
transverse fissure of Bichat (the fissura choroidea). 

The frontal section through the anterior pair of the corpora 
quadrigemina (Fig. 61) shows the three divisions: the crusta, teg- 
mentum, and quadrigeminal ganglia. Toward the outer side is the 
pulvinar, with the lateral geniculate body. Emerging below the pul- 

Corp. genie. 

Ant. corpora med. 
quadrigemina 

I Brachium 
Aquaeductus\ °f™ r pus I ^Bulvinar 



Tegmentum 




t— / Crusta 



JPyramis „ \ 

Corpus 

genie. Tat, 



Post, lonqitud. bundle 



Substantia 



Red nucleus 



nigra 



Fig. 61.— Diagrammatic Cross-section through the Anterior Corpora Quadri- 
gemina. (After Edinger.) 



vinar is the crus, which contains the pyramidal tract. Between it and 
the tegmentum, in which is seen the red nucleus, is situated the sub- 
stantia nigra. Below the aqueduct are the root fibres of the motor 
oculi, and in characteristic transverse section the posterior longitu- 
dinal bundle. The position of the latter is made still clearer in the 
longitudinal section represented in Fig. 62. 

The manner in which the fibres from the red nucleus pass under 
the posterior pair of the corpora quadrigemina toward the middle 
line and then decussate with the fibres of the opposite side — the so- 
called "crossing of the brachia conjunctiva" (sup. peduncles of the 
cerebellum) — is represented in Fig. 63. 

Isolated lesions of the corpora quadrigemina are almost as 
rare as similar lesions of the capsule ; nearly always neighbor- 
ing structures are implicated. The data which we possess in 
this connection seem to indicate that lesions of the anterior pair 
produce visual disturbances, amblyopia, amaurosis, and loss of 



THE CORPORA QUADRIGEMINA. 



20 1 



pupillary reaction. Physiologically important is the fact that 
a root going to the optic tract is given off from this anterior 




) j S Corpora quadrigemina 

V / Aquaeductus . Cu**^ 



Ventr. * 4 










Fig. 62. — Longitudinal Section through the Region of the Corpora Quadri- 
gemina of a Human Foetus Twenty-eight Weeks Old. (After Edinger.) Shows 
that the posterior longitudinal bundle terminates in the nucleus of the oculo-motor nerve. 

pair, and that radiating fibres pass to the nucleus of the third 
nerve, so that a connection exists between stimulation of the 




Fig. 63. — Diagrammatic Horizontal Section through the Decussation of the 
Superior Peduncles of the Cerebellum. (After Edinger.) 



202 



DISEASES OF THE BRAIN PROPER. 



optic nerve and stimulation of the oculo-motor (pupillary re- 
flex) (Mendel). Authors seem to differ, however, about the 
extent to which this reflex is influenced by disease of the ante- 
rior pair of the corpora quadrigemina. Impairment of certain 
movements of the eyes, especially the upward motion of the 
ball, has been repeatedly noted by competent observers (Gow- 
ers). Nothnagel assumes that a lesion of the same oculo-motor 
branches on both sides, without the existence of an alternating- 
paralysis of the extremities, speaks for a lesion of the corpora 
quadrigemina (cf. loc. cit., p. 220). As to the function of the 
posterior pair of the corpora quadrigemina, all explanations are 
uncertain and hypothetical. Baginsky assumed them to have 
a singular significance for the ear, as the anterior pair for the 
eye — an idea in support of which further evidence is needed ; 
and the disturbance of equilibrium which has been ascribed 
to disease of these bodies, and which recently has again been 
studied by Eisenlohr (Deutsche med. Wochenschrift, 1890, 42), 
may well be produced by pressure upon the neighboring ver- 
miform process of the cerebellum. On this point nothing posi- 
tive is known. 

1 opticus 
1>5 ' 




Lemniscus tract 

Pyramidal tract u C 

Fig. 64. — Sagittal Section through Pons and Medulla Oblongata. (After Men- 
del.) /, anterior commissure. _p 4 , pulvinar. s, substantia nigra, r, tegmentum of 
crus cerebri. r x , red nucleus. /, pes pedunculi. u, pons, v, hypoglossus nucleus with 
fibres emerging from it. a u corpus quadrigeminum anterius. <x 2 , corpora quadrigemi- 
num posterius. y, ansa lenticularis. 5, Vicq-d'Azyr's bundle. A, optic tract. \ u exter- 
nal thalamus-root of optic tract. A 2 , internal thalamus-root of optic tract, v, olivary- 
body, o, anterior pyramid, t, posterior longitudinal body, /c, locus caeruleus. v. m, 
valve of Vieussens. m. 6, Meynert's bundle, ad, abducens nucleus with emerging fibres. 
Bd, superior peduncle of cerebellum, oc, oculo-motor nucleus with emerging fibres. 

The crura cerebri emerge from the pons Varolii as two thick 
cylindrical white bundles of fibres ; on leaving it they diverge, 



LESIONS OF THE CRURA CEREBRI. 



20- 



having between them the posterior perforated space and the 
corpora albicantia (mammillaria s. candicantia). The situation 
of the crusta and the tegmentum, and the masses of fibres con- 
tained in them, is once more shown in Fig. 64, which represents 
a longitudinal, sagittal section made almost in the middle line 
(r, tegmentum ; s, substantia nigra ; t, crusta). That the crusta 
forms the path for the voluntary, the tegmentum the path for the 
reflex movements, and that the latter also contains the sensory 
paths, as Meynert assumes, has not yet been proved by physi- 
ology. That the crusta, however, contains the motor path — 
namely, the pyramidal tracts — is a fact established beyond 
doubt ; hence its lesions will for the present be of more prac- 
tical interest. Only a small number of instances of lesions in 
the tegmentum have been reported. A case of Buss (cf. lit.) 
presented ataxia of all 

four extremities, anass- qua. 

thesias, disturbances of 
the muscular sense, and 
an affection of the right 
hypoglossus. At the 
autopsy a focal lesion 
was found in the teg- 
mentum of the crus 
and the pons. 

Considering the re- 
lation which the third 
nerve bears to the me- 
dian part of the crus 
cerebri, as is shown in 
Fig. 65, we can well un- 
derstand that in lesions 
of the latter the oculo- 
motor is not rarely im- 
plicated, and autopsies 
have frequently demon- 
strated that wherever 
an oculo-motor paraly- 
sis has been associated with paralysis of the extremities on the 
opposite side, the lesion is situated in the crus cerebri. For 
example, in a patient with oculo-motor paralysis of the right 
side and hemiplegia of the left side (if both come on at the 
same time !), we may without hesitation diagnosticate a focal 




Fig. 65. — Cross-section through the Region of 
the Anterior Corpora Quadrigemina. gu. a., 
anterior corpora quadrigemina. g. c, gray matter 
around the aqueduct of Sylvius, aq., aqueduct of Syl- 
vius, nlll, nucleus of the third nerve. ///., posterior 
longitudinal bundle, r. k., red nucleus (tegmentum). 
sn, substantia nigra (locus niger). /, cerebral pe- 
duncle. 



204 



DISEASES OF THE BRAIN PROPER. 



lesion in the right crus cerebri ; if, in addition, anaesthesia exists 
on the paralyzed side, an implication of the tegmentum must be 
suspected. Mendel has called attention to the fact that patients 
with tumors of the crura sometimes urinate frequently. How 
far this observation may be taken as confirmatory of the view of 
Budge, who holds that the centre for the secretion of urine is 
situated in the peduncles, future studies will have to teach us. 

The pons Varolii, which connects the two hemispheres of 
the cerebellum, contains, as we have said above, the nuclei for 

several nerves and the fibres passing* 
from them to the brain. The nuclei, 
which are situated in the upper seg- 
ment, are those of the fifth, the facial, 
and the abducens. Since the pons natu- 
rally also contains the motor fibres, situ- 
ated, as we said above, in the lower or 
ventral segment, while in its dorsal part 
one meets the sensory bundles, pontine 
lesions may produce a complication of 
symptoms as characteristic as those fol- 
lowing lesions in the crus. As we have 
attempted to make clear in Fig. 66, the 
fibres of the facial nerve decussate higher 
up than the motor fibres of the pyrami- 
dal tracts. Keeping this fact in mind, 
we can easily understand that a lesion of 
the lower part of the pons concerns the 
facial fibres after their decussation, the 
fibres going to the extremities, howev- 
er, before they cross, and consequently 
gives rise to a facial paralysis on the 
side of the lesion, but a paralysis of the 
extremities on the opposite side (hemi- 
plegia alternans) (Gubler, 1859). A ^ e " 
sion of the upper part of the pons con- 
cerns both of these paths before their 
decussation, and produces, therefore, 
hemiplegia, with a facial paralysis of the same side, which, how- 
ever, is distinguished from the typical hemiplegia in that the 
facial paralysis in this case resembles somewhat the peripheral 
type, as it takes in all three branches of the facial, and as, 
though but rarely, reaction of degeneration may be present. 




Fig. 66.— Diagram showing 
the Decussation of the 
Fibres going to the 
Extremities, and of 
those going to the 
Face, in the Pons and 
Medulla Oblongata. F 
facial fibres. F, fibres go- 
ing to the extremities. P, 
pons. O, medulla oblon- 
gata, pyx, decussation of 
the pyramidal tracts, a, a 
focus in the upper, b, a 
focus in the lower, part of 
the pons (the latter is situ- 
ated below the decussation 
of the facial fibres). 



LESIONS OF THE CEREBELLUM. 



205 



If, then, we meet with a paralysis which affects the facial 
on one, the extremities on the opposite side (alternating paraly- 
sis), simultaneously, we are justified in assuming the lesion to 
be situated in the pons, and more especially in its lower part. 
If paralysis first occurs in the face alone, and does not develop 
in the extremities until later, and if the whole process is grad- 
ual, it may arise from a tumor at the base of the brain. If, be- 
sides the symptoms described, the patient complains of pain in 
the face, the trigeminus is included in the lesion. A paralysis 
of the external rectus points to the implication of the abducens 
nerve, in which case a paresis of the internal rectus of the other 
side not rarely coexists, so that a conjugate deviation of the 
eyeballs toward the paralyzed side — that is, away from the 
focus — may occur. 

Bilateral lesions of the pons must be thought of in com- 
bined paralyses of the extremities and cranial nerves, or in 
cases of bilateral facial paralysis or bilateral paralysis of the 
extremities (either of both legs or of all four extremities). 
The diagnosis, however, as a rule, can not be made with cer- 
tainty. 

Convulsions will be observed if by acute lesions the spasm 
centre, as Nothnagel calls it, becomes excited. Tonic spasms 
in the paralyzed limbs are not uncommon. Anarthric speech 
disturbances in bilateral affections of the pons have been noted 
by Markowski (Inaugural Dissertation, Dorpat, 1890). Psychi- 
cal changes, which occur in connection with lesions in the pons, 
are very irregular in their occurrence, and assume the most 
diversified forms. They deserve a more careful study than has 
as yet been devoted to them. Their entire absence has been 
repeatedly noted. Anaesthesias in the distribution of the tri- 
geminus, as well as in the extremities, are comparatively fre- 
quent, but we are not at present able to utilize them for the 
purpose of topical diagnosis. 

To enable us to point with certainty to the cerebellum as 
the seat of disease, the implication of the vermiform process is 
necessary, since, as Nothnagel has pointed out, we may have 
extensive disease in the hemispheres without the manifestation 
of a single symptom during life. In the cases, however, in 
which the vermiform process is affected, marked disturbances 
of co-ordination and equilibrium ensue ; the patient staggers 
and complains of severe vertigo on walking and standing. 
This is almost a pathognomonic symptom, especially if it be as- 



206 DISEASES OF THE BRAIN PROPER. 

sociated with occasional spells of more or less serious vomiting. 
Since, however, cerebellar ataxia may be absent in tumors of 
the vermiform process (Eisenlohr), we are not surprised that it 
is often very difficult to make a diagnosis. 

Lesions of the middle peduncles of the cerebellum produce 
highly characteristic symptoms, so that a diagnosis can be 
made with a fair amount of certainty. The body is involun- 
tarily gyrated around its longitudinal axis (" forced movement "). 
This symptom, however, can only be observed as a consequence 
of irritation of the peduncles, but is absent if the latter are 
wholly destroyed — e. g., by haemorrhage. Sometimes the pa- 
tient has an irresistible inclination to lie on one side, and this 
is, if the remaining symptoms point in the same direction, also 
to be estimated as a forced movement, or rather a " forced posi- 
tion." It is not uncommonly accompanied by a corresponding 
twist of the head and eyeballs. This phenomenon, however, is 
not a pathognomonic symptom for lesions of the middle pe- 
duncles. The direction in which the body is turned is some- 
times toward the diseased side, sometimes away from it, a fact 
for which no explanation has as yet been found. 

For lesions of the other peduncles of the cerebellum (the 
superior and inferior) no diagnostic points are known. 

The lowest part of the encephalon is called the medulla oblon- 
gata. It becomes continuous below with the spinal cord on a level 
with the lower margin of the foramen magnum. On its anterior 
(lower, ventral) aspect we observe the pyramids with their decussa- 
tion, and the olives, while to the outer side of these are to be found 
the restiform bodies, the inferior peduncles of the cerebellum. The 
last contain the so-called direct cerebellar tracts, which, coming from 
the outermost portion of the lateral columns in the cord, pass, through, 
the anterior commissure of the vermiform process, to the cortex of 
the cerebellum. That a relation exists between the olives and the 
cerebellum is apparent from the fact that wherever we have a con- 
genital atrophy of the cerebellum these bodies are also atrophic 
(Flechsig). 

On the posterior (dorsal, upper) aspect is the floor of the fourth 
ventricle, the fovea rhomboidalis (Fig. 67), which is bounded below 
by the diverging restiform bodies, above by the diverging superior 
peduncles of the cerebellum. The median columns are called the 
posterior pyramids (funiculi graciles). They are the continuations 
of Goll's columns of the spinal cord. To the tracts situated to the 
outer side of these the name funiculi cuneati, or Burdach's columns,, 
has been given. 



THE MEDULLA OBLOXGATA. 



20J 



To diagnosticate the medulla oblongata as the seat of a 
lesion is only possible if the nuclei in the floor of the fourth 
ventricle are diseased, in which case we get the clinical picture 
of bulbar paralysis. Other characteristic symptoms do not 
exist, and more especially it must not be forgotten that foci in 




Fig. 67.— The Connections of the Cerebellum— with a, the midbrain (superior pedun- 
cles), 5 ; b, the pons (middle peduncles), 7 ; c, the medulla oblongata (inferior pedun- 
cles or restiform bodies;, 3. 1, fourth ventricle. 2, striae acusticae. 4, funiculi graciles. 
6, lemniscus. 8, corpora quadrigemina. 

the medulla may give rise to a paralysis only in the extremities, 
which presents nothing characteristic during life. If, however, 
the nerve nuclei of the medulla are implicated, a characteristic 
picture is presented which can hardly be mistaken. Another 
point of which, in making our topical diagnosis, we must not 
lose sight, is the fact that certain brain lesions may give rise 
to a similar combination of symptoms constituting the clinical 
picture of the disease which we have described above as 
pseudo-bulbar paralysis. Other diseases of the medulla — trau- 
matism, acute and gradual compression, haemorrhage, and em- 
bolism — are of no practical significance, since they cause death 
so quickly that a certain diagnosis is impossible. Hence we 
will pass them over without further remark. 

LITERATURE. 

Optic Thalamtis. 

Bechterew. Die Bedeutung der Sehhiigel auf Grund von experimentellen und 

pathologischen Daten. Virchow's Archiv, 1887, ex, Heft 2, p. 322. 
Kirilzew. Zur Casuistik der Sehhugelaffectionen. Med. Oboszenije, 1891, 4. 



208 DISEASES OF THE BRAIN PROPER. 

(Russian. Tumor in the Left Thalamus, with Loss of the Movements for 
Facial Expression in the Opposite Side of the Face.) 
Eisenlohr. Deutsche Zeitschr. f. Nervenhk., 1893, iii, 4, 5. 

Crura Cerebri. 

Brissaud. Degenerations secondaires dans le Pedoncule cerebral. Publications 
du Progres med., 1879. 

Tomaszewski. Zur Pathologie des Grosshirnschenkels. Inaug.-Dissert., 
Breslau, 1881. 

Schrader. Ein Grosshirnschenkelherd mit secundaren Degenerationen der 
Pyramide und Haube. Inaug.-Dissert., Halle, 1884. 

Leube. Deutsches Arch. f. klin. Med., 1887, xl, 2, p. 270. 

Buschke. Zur Casuistik der Herderkrankungen des Hirnschenkels. Inaug.- 
Dissert., Berlin, 1892. 

Bannister. Journ. of Nerv. and Ment. Diseases, 1890, xv, 9. 

Pons. 
Senator. Zur Diagnostik der Herderkrankungen in der Briicke und dem ver- 

langerten Marke. Arch. f. Psych, u. Nervenhk., 1883, xiv, 3, pp. 643 et seq. 
Bleuler. Zur Casuistik der Herderkrankungen der Briicke. Inaug.-Dissert., 

Leipzig, 1885. 
Meyer. Beitrag zur Lehre der Degenerationen der Schleife. Arch, f. Psych, u. 

Nervenhk., 1886, xvii, 2, pp. 439 et seq. 
Markowski. Arch. f. Psych, u. Nervenhk., 1891, xxiii, 2. 
Delbanco. Beitrage zur Symptomatologie und Diagnostik der Geschwulste des 

Pons Varolii. Inaug.-Dissert., Berlin, 1891. 
Porter. Brit. Med. Journ., April 18, 1891. 
Brasch. Zur Casuistik der pontilen Herderkrankungen. Neurol. Centralbl., 

1892, 2. 
Diller (Pittsburg). Amer. Journ. of the Med. Sciences, November, 1892. 
Moeli und Marinesco. Arch. f. Psych., 1892, xxiy, 3. 
Kolisch. Wiener klin. Wochenschr., 1893, 14. 
Starr. New York Med. Record, February 6, 1893, xliii. 

Cerebellum. 
Krauss. Ueber Tuberkelknoten des Cerebellum. Inaug.-Dissert., Berlin, 1888. 
Bressler, Frank C. Abscess of the Cerebellum following Suppurative Otitis 

Media. Philadelphia Med. Times, August 1, 1888. 
Gowers. Lancet, 1890, i, 18, p. 955. (Functions of the Cerebellum.) 
Bohm. Ueber cerebellare Ataxie nebst einem casuistischen Beitrage zur Lehre 

von den Kleinhirngeschwiilsten. Inaug.-Dissert., Strassburg, 1891. 
Cramer. Beitrage zur pathologischen Anatomie und allgemeinen Pathologie. 

Jena, 1891, xi, 1. (Unilateral Atrophy of the Cerebellum.) 
Luciani. Das Kleinhirn. Leipzig, Besold, 1893. 

Medulla Oblongata. 
Spitzka. Deutsche med. Wochenschr., 1887, 8, p. 157. (Focal Lesions at the 

Level of the Transition from the Pons to the Medulla Oblongata.) 
Goldberg, Ludwig. Tumoren der Oblongata. Inaug.-Dissert., Jena, 1889. 



PA THOL O GICA L DIA GNO SIS. 



209 



II. The Study of Cerebral Lesions with Reference to 
their Pathological Nature. 

Pathological Diagnosis. — We have before pointed out that 
the question as to the nature of a brain disease is not only of 
interest to the physician, but of the greatest importance to the 
patient, as on this the prognosis as well as the mode of treatment 
turns. An error in the topical diagnosis may deserve the cen- 
sure of scientific criticism, but does not necessarily entail dam- 
age to the patient. If, on the other hand, we mistake the nature 
of the lesion in a given case — if, for instance, a disease of the ves- 
sels is taken for a new growth, if the tuberculous or syphilitic 
nature of the affection is overlooked, or, again, a severe alcoholic 
intoxication is diagnosticated where in reality an apoplexy exists 
— when such errors have influenced the treatment, not only op- 
portunities may be lost for the patient which may never present 
themselves again, but an unfavorable event of the disease may 
actually be brought about or at least precipitated. On these 
grounds we ought to be particularly careful and conscientious 
in forming this part of our diagnosis, and no symptom, how- 
ever small it may seem, should be overlooked, as we never know 
but that it may later perhaps become of diagnostic value. 

In looking over the several pathological processes which here 
concern us, we find that their number is comparatively limited. 
First of all, we shall devote our attention to diseases of the blood- 
vessels, which so frequently are the cause of cerebral lesions. 
We shall have to determine the nature of these diseases, and 
carefully distinguish the affections of the blood-vessels from the 
secondary changes produced by them. The clinical symptoms, 
the complaints of the patient, and the objective signs are a 
direct consequence of the latter only, and it is therefore not the 
disease of the blood-vessels which we have practically to deal 
with, but the changes in the brain substance which they entail. 
The clinical manifestations vary according to the seat of the dis- 
eased vessel and the portion of the brain supplied by it. The 
symptoms we shall describe in detail later; but first let us speak 
of the pathological nature of the diseases of the cerebral vessels. 

AFFECTIONS OF THE BRAIN DUE TO DISEASE OF THE BLOOD-VESSELS. 

A. Diseases of the Cerebral Vessels and their Conse- 
quences. — The arteries of the brain are derived from the internal 
carotids and the basilar, which is formed by the two vertebrals. 
14 



2IO 



DISEASES OF THE BRAIN PROPER. 



The internal carotid gives off two terminal branches, the anterior 
cerebral (arter. corpor. callos.) and the middle cerebral (arter. foss. 
Sylv.). The basilar divides into the two posterior cerebrals (arter. 
profund. cerebri). These receive on each side a communicating 
branch from the internal carotid, the so-called posterior communi- 
cating artery, while the two anterior cerebrals are connected by an 
anterior communicating branch, so that a closed circle (or rather a 
heptagon, according to Hyrtl) of arteries is formed, known as the 
circle of Willis, an arrangement which is of the last importance for 
the distribution of the blood in the brain (cf. Fig. 68). 




Fig. 68. — Diagram showing the Circle of Willis. The carotids with the anterior and 
middle cerebral arteries and the basilar with the posterior cerebrals are connected by 
communicating branches. 



The fact that the left carotid comes off from the aorta nearly in 
a straight line with the blood-current in the arch, while the innomi- 
nate, which gives off the right carotid, leaves the aorta almost at 
right angles, easily explains the greater frequency of embolism on 
the left side. A somewhat similar condition exists in the vertebrals, 
where the left, often the larger one, arises from the subclavian at its 
highest point. This is, however, of less moment for cerebral lesions, 
as the blood has first to pass the basilar before entering the brain- 
substance. 



THE CEREBRAL VESSELS. 



211 



Of the three before-mentioned arteries — the anterior, middle, and 
posterior cerebrals — each one supplies two sets of vessels totally dis- 
tinct from each other — namely, first, the so-called cortical arteries ; 
second, the arteries of the basal ganglia. The important difference 
between these two systems consists in the fact that the former, as 
Heubner and Duret have shown, possess anastomoses, while the latter 
are, as they have been called by Cohnheim, terminal arteries — that is, 
they do not communicate with each other, but pass directly into the 
capillaries. The significance of such an arrangement is apparent, and 
we shall not be surprised to find that occlusion of an artery of the 
second set almost always produces death of the parts supplied by it. 

Of the three cerebral arteries, the middle, the Sylvian artery, has 
by far the widest distribution and is the most important ; for while 
the anterior supplies the corpus callosum, the gyrus rectus, the para- 
central lobule, and the precuneus ; the posterior, the crus, the tem- 
poral, and the occipital lobe, and the cuneus, sending also a few 




Fig. 69.— The Cortical Distribution of the Middle Cerebral Artery. (After 
Charcot.) From left to right the five branches are named as follows: The inferior 
frontal branch to Broca's convolution, the ascending frontal branch, the ascending parie- 
tal branch, the parieto -sphenoidal, and the sphenoidal branches. 

branches to the optic thalamus (arter. optic, posterior.), it remains for 
the middle cerebral to supply the whole lenticular and the caudate 
nucleus, and, above all, the internal capsule. Moreover, the central 
and cortical motor region, the cortical areas concerned in the process 
of speech (on the left side), the cortical centre for hearing, probably 
also for vision, depend on this artery for their nutrition. 

Its cortical distribution, its subdivision into the frontal, parietal, 
parieto-sphenoidal, and sphenoidal arteries, is made clear by Fig. 69. 



212 



DISEASES OF THE BRAIN PROPER. 



Its distribution to the lenticular nucleus is illustrated in Fig. 70. 
The internal artery of the corpus striatum, also called the lenticular 
artery, goes to the first and second segment of the lenticular nucleus, 
while the external branches are the so-called lenticulo-striate and 
lenticulo-optic arteries. Among the former, the one which supplies 
the third segment of the lenticular nucleus, the upper portion of the 
internal capsule, and the caudate nucleus deserves special mention. 
It is so frequently the seat of haemorrhage that Charcot has called it 
"Vartere de V hemorrhagic cere'brale." Mendel has attempted to show 




Fig. 70.— Frontal Section through the Cerebral Hemispheres, One Centime- 
tre behind the Chiasm. Shows the distribution of the middle cerebral artery in the 
lenticular nucleus. 



experimentally the physical reasons why ruptures are especially prone 
to occur at this place (Berliner klin. Wochenschr., 1891, 24). The ac- 
count of these experiments and the discussion which followed their 
presentation at the Berlin Medical Society, in the session of May 27, 
1897, are well worth reading (Deutsche Med.-Zeitg., 1891, 46). 

The 'tween-brain and the mid-brain are mostly supplied by the 
posterior communicating and its branches, the cerebellum by several 
so-called cerebellar branches (arter. cerebell. super, et infer.) coming 
from the vertebrals ; the pons and medulla oblongata also by branches 
of the vertebrals, which are the so-called rami ad pontem and rami 
ad medullam oblongatam. 

The internal carotid and the basilar measure 4 mm. in diameter ; the 
vertebrals, 3.5 mm. (Luschka). The blood pressure in the carotid is gen- 
erally taken to correspond to from 140 to 160 mm. Hg. How guarded, 
however, we ought to be in accepting such statements has been shown 
by Loewenfeld, who drew attention to the variations in the development 
of the cerebral arteries; and it seems at least possible that this is of 
considerable aetiological significance for different cerebral- affections. 



CEREBRAL HEMORRHAGE. 



LITERATURE. 



213 



Capelli e Brugia. Arch, di psichiatria, science penale, etc., 1886, Fasc. 1. (On 
the Action of Certain Drugs upon the Cerebral Circulation.) 

Loewenfeld. Arch. f. Psych, u. Nervenkrankh., 1887, xviii, 5, 

Geigel. Virchow's Arch., 1890, cxix, Heft 1. 

Geigel. Die Mechanik der Blutversorgung des Hirns, Stuttgart, Enke, 1890. 

Lewy. Die Regulirung der Blutbewegung im Hirn. Virchow's Arch., 1890, 
cxxii, 1, 2. 

Mendel. Neurolog. Centralbl., 1891, 24. 

Kolisko. Ibid., 1892, 16. (On the Blood Supply of the Brain.) 

Grashey. Experimentelle Beitrage zur Lehre von der Blutcirculation in der 
Schadel-Riickgratshohle. Festschrift, Miinchen, 1892. 

I . Cerebral Hemorrhage, Hezmorrhagia Cerebri {Periarteritis 

Cerebralis, Miliary Aneurisms of the Cerebral Arteries). 

Pathological Anatomy and ./Etiology. 

Of all cerebral affections, hsemorrhage, the result of the 
rupture of a vessel, is by far the most important and the most 
frequent. As we should expect, haemorrhages of various kinds 
may be produced by traumatism (injury to the skull, with or 
without fracture). They may occur between the inner side of 




Fig. 71. — Cerebral Artery from an Apoplectic Focus, ma, miliary aneurism. ble y 
extravasations of blood into the adventitial lymph space. (After Cornil and Ranvier.) 

the skull and the loosened dura mater, or in the sac of the dura 
or that of the pia (submeningeal hsemorrhage) ; but, disregard- 
ing these, there is one affection especially which gives rise to 
cerebral haemorrhage — namely, a diffuse periarteriitis — which 




214 DISEASES OF THE BRAIN PROPER. 

was first described by Charcot and Bouchard in 1868. In this 
process a thickening of the lymph-sheaths and subsequent 
changes in the muscularis take place, by which the formation of 
miliary aneurisms is favored (cf. Figs. 71 and 72.) Rupture of 

these aneurisms then gives 
rise to haemorrhages, and 
so frequently is this the 
case that the authors be- 
fore mentioned found this 
condition in every one of 

Fig;. 72. — Miliary Aneurism of a Small , ,• 

6 A ' T ^ T seventv -seven consecutive 

Artery of the Lenticular Nucleus. * \ ^uuo^uuvv 

(After marchand.) cases which came under 

their observation. 

For the rupture of these aneurisms it is by no means always 
necessary to have any extraordinary exciting cause, such as an 
elevation of the blood pressure, which may temporarily be pro- 
duced by bodily exertion, sneezing, coughing, vomiting, and the 
like, or which may permanently exist where the heart is hyper- 
trophied, as in valvular disease or in cases of contracted kid- 
ney. In many of the instances nothing of the kind can be 
demonstrated. 

The size of the aneurisms varies from 0.2 to 1 mm. ; their 
color and consistence often differ greatly. Their favorite seat 
is in the thalamus, the corpus striatum, the convolutions, and 
the pons, while they are less frequently met with in the cen- 
trum ovale, the crura, and the medulla oblongata. Sometimes 
only two or three, at other times as many as several hundred, 
have been detected in one brain. On being squeezed they are 
found to contain white corpuscles, fat droplets, and amorphous 
granular masses. 

The haemorrhage which is produced by their rupture con- 
sists when fresh of a dark-colored loose coagulum. The wall 
of the " focus " is red and sported with punctiform haemor- 
rhages (capillary apoplexies), and presents a ragged and torn 
appearance. Gradually the dark color becomes lighter, the 
neighboring parts are infiltrated, yellowish, and very soft 
(lemon-colored oedema). As a rule, the focus is later en- 
capsuled by a layer of neuroglia, the fibrin masses become 
mixed with the debris of the nerve elements, and we get a 
smooth-walled cavity with liquid contents, the so-called apo- 
plectic cyst, occupying a smaller space than the original 
haemorrhage. If the walls approach each other before the 



AETIOLOGY OF CEREBRAL HEMORRHAGE. 



215 



coagulum is transformed, a great increase of fibrillated con- 
nective tissue takes place and we get a so-called apoplectic 
scar. 

The effect of the haemorrhage varies according to its posi- 
tion, according to the calibre of the ruptured vessel, upon 
which depends its amount, and according to the rapidity or 
slowness with which the blood escapes. The favorite seat for 
haemorrhages is in the large ganglia (Charcot, Andral, Ro- 
choux) ; with decreasing frequency they are found (Noth- 
nagel) in the remaining portions of the cerebral hemispheres, 
much more rarely in the pons and the cerebellum. The fre- 
quency is directly influenced by the size of the different cere- 
bral vessels and by the blood pressure. The diameter of the 
vessels of the brain stem is considerably larger than that of 
those going to the cortex. The above-mentioned " artery of 
cerebral haemorrhage " is of an especially large calibre (i x / 2 
mm.), and causes therefore when it bursts a particularly large 
and extensive haemorrhage, because the bleeding is prolonged. 
" The traumatic effect of the haemorrhage," as Wernicke calls 
it, is equal to the product of the mass of effused blood into the 
square of the rapidity with which it is poured out, which latter 
depends directly upon the blood pressure in the vessels. 
Hence it follows that, as regards the effect of a haemorrhage, 
the blood pressure is of more importance than the calibre of 
the vessel. 

^Etiology. — In examining into the aetiological factors con- 
cerned in a cerebral haemorrhage, we must distinguish those 
which produce the disease of the vessels from those which 
directly cause the haemorrhage ; in other words, the predis- 
posing from the exciting causes. 

About the former not much is known ; nevertheless, con- 
siderable influence in the causation of arterial disease must be 
ascribed to age, as we can not deny that it is decidedly less 
frequently to be observed in the young than in older persons, 
and that the smallest percentage of apoplexies is found between 
the fifth and thirtieth years of life. Still, to lay so very much 
stress upon the significance of age is not warranted by experi- 
ence. The fact that cerebral haemorrhage is bv no means rare 
in people from twenty to thirty years old clearly shows that 
miliary aneurisms may occur even at a comparatively early 
period of life ; nor are these cases by any means always those 
of persons laboring under hereditary disadvantages, since even 



2i6 DISEASES OF THE BRAIN PROPER. 

members of perfectly healthy families, while still young, may 
fall victims to a stroke of apoplexy. The influence of heredity 
as well as that of age has undoubtedly been overrated in this 
connection. It is true there are families in which apoplexy 
seems to be a natural occurrence, but such instances are excep- 
tional, while on the other hand the arterial disease develops in 
an infinitely larger proportion of cases apparently without 
special hereditary cause. Sometimes the development of the 
disease seems to be favored by a peculiar "habitus" Thus, 
corpulent individuals of medium height, with short necks, 
broad thoraces, who on the least exertion or excitement become 
purple in the face, have usually been looked upon as particu- 
larly predisposed to apoplexy, and in many cases with justice ; 
yet those who have in an extensive practice seen how often tall, 
spare individuals with narrow chests die from cerebral haemor- 
rhage, will readily give up the idea that an apoplectic habitus 
is a conditio sine qua non. 

The role which sex plays can not be denied. The disease is 
much more frequently observed in males than in females, while 
with embolism, as we shall see, the reverse is true. To explain 
this predisposition in males, other factors — namely, the mode of 
life — must, I think, be taken into account, and here it is, in the 
first place, the occupation, and, secondly, the abuse of alcohol, 
which must be considered. Notwithstanding the fact that we 
know very little about the influence of occupation on the for- 
mation of miliary aneurisms, our statistics of fatal cases of cere- 
bral haemorrhage in the different trades being somewhat unre- 
liable, still we have some sure grounds, the correctness of 
which can scarcely be called in question. That, for instance, 
the working in certain poisons, especially in lead, predisposes 
to arterial disease, and consequently to apoplexy, is indisputa- 
ble. In his thesis on encephalopathia and arthralgia saturnina, 
prepared under my auspices, Schulz (Breslau, 1885) points out 
the frequency of the so-called hemiplegia saturnina, and calls 
attention to the fact that Berger has made similar observations. 
In the second place, those who are exposed to radiating heat- 
workers at furnaces, puddlers — are in danger, especially if their 
work is connected with much bodily exertion, and this can 
hardly surprise us if we remember how much circulatory dis- 
turbances are favored by such circumstances. The same may 
be said of occupations which necessitate uncomfortable posi- 
tions of the body, as, for instance, is the case in agate polishers, 



ETIOLOGY OF CEREBRAL HEMORRHAGE. 



21/ 



who constantly have to lie on their abdomens, or in coal miners, 
who have to remain in a stooping position all the time. 

In regard to the abuse of alcohol we refer not only to the 
confirmed drunkards, but much rather to that class of indi- 
viduals who habitually consume more alcohol, especially beer, 
than is good for them. Such men rarely, if ever, get drunk, 
but they drink several times a day one or two glasses of beer, 
do not take enough exercise, and become fat and predisposed 
to fatty heart and arterial disease, especially arterio-sclerosis, 
which affection, we may say finally, is the real cause of the 
greater frequency with which apoplexy is met with in men 
than in women. The fatty heart may be present even without 
any marked obesity. 

The important influence of syphilis in the origin of cerebral 
haemorrhage is proved by many irrefutable observations, and, 
considering the part played by it in disease of the cerebral 
vessels, this can easily be explained. We shall mention it 
again in this chapter, and later dwell more particularly on the 
symptoms peculiar to the syphilitic hemiplegia. Exceptionally, 
hemiplegia occurs after diphtheria, sometimes in conjunction 
with a paralysis of the palate, sometimes independently. In a 
girl aged fifteen under my care, hemiplegia developed fourteen 
days after diphtheria without any simultaneous disturbance of 
consciousness, and only slight improvement was noticed after 
several years (cf. also Seifert, Neurolog. Centralblatt, 1893, 4). 
With equal rarity is this complicating sequela found after 
other acute diseases — for instance, scarlatina. 

Sometimes no exciting cause can be demonstrated, but if 
such be observed, they are always associated with a sudden 
more or less marked increase of the blood pressure. People 
with diseased cerebral vessels are not rarely suddenly attacked 
by an apoplectic stroke after strong emotion, hard bodily exer- 
tion, during violent attacks of coughing, sometimes also in a 
cold bath and after a full meal. Christian (Arch, de Neurol., 
1889, 53), and Bollinger in his monograph on late traumatic 
apoplexy (Festschrift fiir R. Virchow, 1891), have pointed out 
that traumatism may also lead to apoplexy. 

How it comes about that the coldest months of the year 
yield the largest percentage of victims of apoplexy, and why it 
is that in the twenty-four hours there are two periods with a 
maximum and a minimum death-rate, if such be actually the 
case, can not be explained. Such has, however, been claimed 



218 DISEASES OF THE BRAIN PROPER. 

by Sormani, who based his statements upon an extensive study 
of statistics (Riv. clin., ser. 2, i, 12 Dicembre, 1871). The same 
author is also inclined to attribute to the barometric pres- 
sure some influence on the mortality, as in his opinion sudden 
changes in the weather materially increase the mortality from 
apoplexy. 

Symptoms and Course. — The rupture of a fair-sized cere- 
bral vessel is always, no matter what part of the brain is affected 
by it, attended with more or less violent symptoms. 

Only in exceptional cases is it preceded by premonitory 
indications (prasmonitorium apoplecticum of Boerhaave). Oc- 
casionally there are temporary sensory disturbances in the 
extremities of one side, formication, numbness, a feeling of 
heaviness in the limbs, pain in the soles of the feet, certain 
choreiform movements in the face and arms (hemichorea prse- 
hemiplegica, Raymond), symptoms which indicate that things 
are not going in their usual order. The patient may also com- 
plain of headache and a feeling of fullness in the head, which 
makes itself manifest on the least provocation, on the slightest 
emotion, or after a small amount of wine has been taken. But 
rarely enough are such premonitions sufficiently appreciated 
by the patient, and only too often are they incorrectly inter- 
preted by the physician. Usually they are overlooked, and are 
first remembered when the catastrophe is either imminent or 
has already taken place. 

When the attack does come on, the patient gradually or 
suddenly loses consciousness, and remains in this condition for 
a few minutes, hours, or even for a day or two, according to 
the severit}' of the " stroke." The higher the blood pressure, 
and the greater the rapidity with which the blood escapes, the 
more pronounced and severe are the general symptoms, which 
collectively are called "apoplectic stroke " (the "insult" of the 
Germans). The way in which the disturbance of conscious- 
ness comes on varies very widely in different cases. Thus one 
patient may for some hours before the actual attack present a 
peculiar excitement, he is restless and bewildered, may even 
have forgotten the ins and outs of his own house, his speech is 
agitated, etc. ; another patient may complain of headache and 
vertigo ; a third of a feeling of heat in his head and of general 
prostration (" different forms of delayed stroke "). All these 
premonitory symptoms which we have described may, how- 
ever, be absent, and a person apparently enjoying the best of 






THE APOPLECTIC ATTACK, 



219 



health may suddenly, as if " struck by lightning," sink to the 
ground and lie there unconscious {apoplexie fondroyante). 

If we are called to such a case, the following conditions will 
present themselves to us on our first examination : The patient 
lies on his bed as if asleep ; his respiration is either quiet and 
deep or loud and stertorous ; he can not be aroused in any way, 
not even by strong irritation of the skin (pricking, tickling) ; his 
eyes are closed, and the pupils, usually of medium size, neither 
much dilated nor much contracted, have lost their power to 
react. With every expiration the cheeks are slightly puffed 
out, and it is often soon apparent that one corner of the mouth 
is lower than the other. The extremities are relaxed, and when 
raised drop loosely. The tendon reflexes are absent in severe 
cases, and neither the cremasteric nor the plantar reflex can be 
obtained. The pulse is full, somewhat slow ; the temperature 
normal, perhaps slightly subnormal ; the urine presents no 
changes, or may contain a trace of albumin, rarely of sugar. 

This condition may, as we have said before, last several 
minutes, several hours, or even one or two days. It is modi- 
fied gradually according as the haemorrhage sooner or later 
comes to a stop or continues without interruption until a fatal 
result ensues. In the former case the patient gradually begins 
to react to strong stimuli, and may open his eyes for a short 
while, when called loudly or when water is thrown over him ; 
he may give a loud yawn and show some voluntary motion 
of the extremities. Gradually consciousness returns, and the 
patient attempts to make himself understood by gestures and 
words, and in the most favorable instances, which are, how- 
ever, unfortunately very rare, the physician can feel assured 
that everything has cleared up, that the patient is again in pos- 
session of perfect consciousness, of the power of speech, and of 
motion. In such cases the " general " symptoms have disap- 
peared without leaving behind any of those belonging to the 
second class, namely, the so-called focal symptoms (Grie- 
singer), and we speak of a "stroke without focal symptoms." 

But the bleeding may continue, although only under low 
pressure, and only cease very gradually ; then the symptoms 
abate but slowly and the recovery is only partial ; the patient 
lies for days in a state of somnolence, and repeated examina- 
tions show that one corner of the mouth is distinctly lower 
than the other, and that the saliva dribbles from it involunta- 
rily. If we can, by strong stimuli, evoke spontaneous move- 



220 DISEASES OF THE BRAIN PROPER. 

ments, it becomes evident that only one side is moved, that only 
one arm or one leg is raised, while the other side remains per- 
fectly motionless, and after consciousness is fully restored the 
certainty is forced upon us that one side of the body is de- 
prived of its power, or, as we say, is paralyzed. This we calL 
a " stroke with focal symptoms." At this stage, however, we 
are unable to decide whether the focal symptoms are the result 
of a destruction of nerve paths or centres — in other words, 
whether they are direct and therefore incurable — or whether 
they depend upon indirect action, so that the loss of function is 
only temporary. If the latter be the case, we speak of " indi- 
rect " focal symptoms. 

Again, the haemorrhage may not cease at all, but continue 
with increasing blood pressure ; then the patient remains un- 
conscious, the breathing becomes irregular and more rapid, 
and assumes the so-called Cheyne-Stokes type, the pulse be- 
comes more rapid and small in volume, the face grows pale 
and haggard, the saliva getting into the trachea produces the 
well-known tracheal rattling, the temperature rises gradually 
but noticeably, and the patient dies without having come to 
himself, and after a period of unconsciousness which may have 
lasted many hours or even several days and nights. 

If, in the course of a " delayed stroke," the breathing, until 
now quiet and regular, suddenly gives place to a rapid, irregu- 
lar, stertorous respiration, and if at the same time the partial 
unconsciousness deepens into a profound coma, the reflexes 
become lost, and tetanic convulsions of the whole body and 
hemicontracture of the paralyzed side make their appearance, 
then we can assume that the haemorrhage has burst through 
into a ventricle, and give an absolutely unfavorable prognosis, 
because in a few hours, more rarely in one or two days, death 
almost invariably follows. The haemorrhage itself in such 
cases is, as can be demonstrated at the autopsy, generally by 
no means copious, but the fact that it is found, even if the 
ependyma of the ventricles be thickened and hardened, speaks 
most clearly for the high arterial pressure under which the 
blood escapes (Wernicke). The bursting of the blood into the 
fourth ventricle is the most rapidly fatal, and it is in these cases 
that we sometimes observe nystagmus. 

The disturbance of consciousness in its many gradations, 
from the slight vertigo to the deep coma, is the most character- 
istic, or at least the most important, symptom of an apoplectic 



APOPLECTIC EQUIVALEXTS. 221 

attack produced by hemorrhage, and it ought not to be under- 
rated, even if it does not become fully developed, but only 
amounts to a transient slight speech disturbance, accompanied 
by a feeling of faintness and weakness. There are patients in 
whom such disturbances occur several times before the onset 
of a real attack. Such patients complain of transient vertigo, 
sligrht weakness, and heaviness in one or the other hand or 
foot; they can at times not find the right word, the correct 
expression, or lose speech entirelv for a short while. All these 
indications are premonitions, not direct forerunners of the 
attack, but symptoms which warn us, indicating that the brain 
is subject to alterations in the blood pressure, a condition which 
may lead to serious consequences if the arterial walls are dis- 
eased (" apoplectic equivalents"). 

Complete absence of all disturbance of consciousness is a 
rare exception, and can only be found when the blood escapes 
quite slowly, so that the increased pressure rises only very 
gradually, and to no great degree. The patient then is seized 
with a sudden weakness, purely physical ; he sinks into a chair, 
and after a few moments, during which time there is not the 
slightest disturbance of consciousness, he becomes aware of a 
sort of difficulty in moving the extremities of one side, which, 
in the most unfavorable instances, in a short time passes into a 
genuine paralysis of that side (focal symptoms without stroke). 
Here may also be mentioned the cases observed by Romberg, 
Graves, Andral, Senator, and others, in which after a hemi- 
plegia no trace of haemorrhage was found at the autopsy, but 
only a diffuse hyperemia of the brain could be demonstrated — 
" pseudo-apoplexy." 

On the other hand, it is not a very unusual occurrence that 
a patient awakening in the morning after a quiet night's rest 
finds himself paralyzed on one side ; in such cases we are, of 
course, not able to decide how much his consciousness would 
have been impaired had he been awake. 

In every hemiplegia that occurs in the course and as a con- 
sequence of cerebral hemorrhage there is a possibilitv of re- 
generation to a greater or less extent : but whether this regen- 
eration will take place, and when, and, moreover, whether it 
will be complete or not, are questions that can not at once be 
decided. They all depend on the condition of the cortico-mus- 
cular tract, as we have pointed out before — upon whether this 



222 DISEASES OF THE BRAIN PROPER. 

be actually interrupted, whether its fibres in places, for instance 
at the internal capsule, be completely destroyed, or whether 
their function be only temporarily impaired in consequence of 
the increased blood pressure, so that after the cessation of the 
haemorrhage a restitutio in integrum of the nerve tissue can 
follow. In the latter case the paralysis disappears after a few 
hours or days, while after an actual interruption of the cortico- 
muscular tract the hemiplegia is incurable, and the patient is 
deprived of the free use of the affected limbs, and, even though 
he may regain after a long time some power of motion, his 
movements will always remain awkward and restricted. 

Sometimes, and this is not very rare, a patient may have 
an apoplectic stroke after which the paralysis disappears 
quickly and entirely, but which is in a few days, on some 
slight provocation, followed by a second attack, accompanied 
by a severe permanent hemiplegia, which under certain cir- 
cumstances can cause death. Such a possibility should always 
be thought of, and we would here say that the prognosis, no 
matter how slight and favorable the apoplexy may seem, should 
always be very guarded. 

Among the " concomitant symptoms " which only excep- 
tionally persist for any length of time, and ought therefore to 
be regarded as indirect focal symptoms, may be mentioned a 
peculiar deviation of the eyes and the head — the " deviation con- 
juguee" of Prevost — generally toward the side of the lesion, so 
that the eyes " look toward the disease-focus." This has been 
thought to be associated with a lesion in the upper parietal 
lobule. Prevost and Landouzy gave this rule : " Le malade 
regarde son hemisphere altere s'il y a paralysie — il regarde ses 
membres convulses s'il y a excitation " (the patient looks to- 
ward the damaged hemisphere if he have a paralysis ; if there 
be irritation he looks toward the convulsed limbs). This is 
seen, for instance, in the so-called cortical epilepsy, which we 
have spoken of on page 187. The head seems forcibly turned 
to one side, and the eyes are turned so far over to the canthus 
that we are scarcely able to test the condition of the pupil ; 
along with this symptom there is found almost always a more 
or less marked dullness of the sensorium. Why this condition 
is generally transient is explained by the fact that the muscles 
of the eyes and neck can be innervated from both hemispheres, 
so that even if one side becomes incapable of working, the 
other can act vicariously for it. Only in bilateral hasmor- 



THE CEREBRAL HEMIPLEGIA. 223 

rbages which produce a permanent paralysis of the eye mus- 
cles is conjugate deviation found to persist. 

Unilateral oculo-motor paralysis on the side of the hemi- 
plegia is very rare ; it is supposed to be associated with lesions 
of the lower parietal lobule. 

After a severe attack there may be a transient polyuria last- 
ing for one or two days ; the specific gravity of the urine, 
which is then faintly acid, may be 1.003 or J-002; at times, but 
not always, albumin or a trace of sugar can be demonstrated 
(Loeb, Prager med. Wochenschr., 1892, 50). This some authors, 
among them Ollivier, were inclined to attribute to an action 
on the centres situated in the floor of the fourth ventricle, the 
existence of which Claude Bernard had already demonstrated. 
This polyuria after an apoplexy does not persist, while this 
may be the case in tumors of the posterior fossa, in focal lesions 
of the pons or the medulla oblongata, where it has to be looked 
upon as a focal symptom (Kahler, Zeitschr. f. Heilk., vii, 2, 3, 
1886). 

In proceeding to the examination of a fresh hemiplegia — 
that is to say, one of a few days' or weeks' duration — the fol- 
lowing points must be borne in mind : 

The facial and the hypoglossal nerve deserve the most 
attention (cf. also Koenig, Deutsche Med.-Zeitg., 1892, 25, p. 
298). The former is injured in its central course, and shows a 
paralysis or only a paresis in its lower branches, while the up- 
per branch is intact ; the patient is unable to inflate the para- 
lyzed cheek, and can not whistle, while wrinkling of the fore- 
head on the paralyzed side presents no difficulty. Careful 
examination shows distinctly that the disturbance on the para- 
lyzed side of the face is much more marked on attempting 
voluntary movements of one side alone, whereas those of ex- 
pression — for instance, laughing, crying — are at least passably 
executed. This, again, may be explained by the fact that mus- 
cles used involuntarily are innervated from both hemispheres. 
The duration of the facial paralysis varies ; sometimes the 
difference between the two sides of the face disappears almost 
completely in a few days, while in other instances it may be 
noticeable for weeks or, in rare exceptions, even during the 
whole life. In this point it resembles the speech disturbance 
caused by a lesion of the hypoglossus, a disturbance consisting 
essentially in faulty articulation, which is noticed by the patient 
more than by those who converse with him. It may disappear 



224 DISEASES OF THE BRAIN PROPER. 

in a few hours, but may persist for months, even years, when 
improvement in the affected side has gone on for a long while, 
and gratifying progress has already been made. A paralysis 
of the same nerve, or rather of the genioglossus muscle sup- 
plied by it, is also responsible if the patient is unable to pro- 
trude the tongue straight ; it is deviated to the paralyzed side 
because the well genioglossus is stronger than the diseased one, 
and consequently pushes the tongue over toward the side of 
the latter. 

The condition of the soft palate is not the same in all cases. 
The velum may be considerably lower on the paralyzed than 
on the well side, but it may also occupy its normal position. 
The uvula is at times deviated to the well, at times to the 
paralyzed side, and again at other times its position may be 
unchanged. These changes do not give rise to any noticeable 
disturbance of function. 

Examination of sensibility in the first few days reveals de- 
cided alterations. Sensibility to pain in most cases is dulled, 
and sensibility to touch and pressure is decreased, though to 
a less marked degree. The patient feels a pin prick either not 
at all on the affected side or, at any rate, with less acuteness. 

Of the nerves of special sense, it is especially the optic which 
takes part in the disturbance. The apoplectic attack may be 
followed under certain circumstances by hemianopia of the 
corresponding side (Gowers), often, too, by amblyopia. 

Smell and taste, as a rule, do not suffer to any great extent ; 
but there is a decrease in hearing power, so that the patient is 
no longer able to understand words spoken in an ordinary tone 
at a distance of fifteen or twenty feet. Such a decrease is not 
rare, yet an absolute (unilateral) deafness never seems to follow 
as a result of an apoplectic attack. 

With regard to mobility, examination shows that either 
the extremities of one side of the body are completely para- 
lyzed (hemiplegia) or that the power of movement in them is 
impaired (hemiparesis). In the latter case the arm is usually 
more affected than the leg and the hand more than the arm. 
Indeed, the movements in the shoulders and elbow joint may 
be as good as normal, while those of the fingers are very awk- 
ward ; in such cases the leg can generally be moved quite well. 
The muscles of mastication and those of respiration are, for 
the reasons above mentioned, almost intact, the muscles of the 
trunk are only slightly implicated, and, if at all, the change is 



THE CEREBRAL HEMIPLEGIA. 



225 



only apparent in the trapezius, so that the shoulder of the 
affected side is raised less energetically than its fellow. 

The tendon and skin reflexes are, in the first few days after 
the attack, decreased or even lost on the affected side, a condi- 
tion which, as we shall see shortly, soon becomes materially 
changed. 

The sensorium usually clears up in from one to four days, 
especially in light cases. The patient again becomes conscious 
of his surroundings, and recollects quite well all incidents which 
happened nearly up to the time of the attack. Thence on, there 
is, of course, a blank in his mind. On awakening, at first he 
has no idea of what has happened to him. His frame of mind 
varies according to the degree of his bodily helplessness, but, 
as a rule, is better than we might expect, considering the 
damage which has been done. Sleep is for weeks much inter- 
fered with. The patients are extremely restless; they throw 
themselves about in bed, and are unable to remain in one posi- 
tion for any length of time. 

The further course depends upon whether the hemiplegia 
proves to be an indirect or a direct focal symptom. 

The slighter cases of indirect hemiplegia, when they have 
not completely passed off after several weeks, are at any rate 
generally improved. The one-sidedness of the face, seen at 
the beginning, has disappeared ; the tongue is now protruded 
straight, speech is again normal, the leg can be moved almost 
as freely as ever, and the only thing which is left as a reminder 
of the dangers through which the patient has passed is a cer- 
tain awkwardness in the movements of the affected hand. 

The graver cases of indirect hemiplegia need from tw r o to 
three months for complete recovery. For weeks after the at- 
tack the patient presents marked disturbances in motion as weil 
as Sensation, and only painfully and with the help of a stick can 
he hobble about his room, while the arm and hand are almost 
useless. Yet a constant progressive improvement of the par- 
alyzed limbs enables us to recognize the favorable tendency of 
the case and to predict with certainty a complete recovery. 

In cases of direct hemiplegia also the course of the disease 
may assume many varieties. All are characterized by the per- 
sistence of the focal symptoms. The attack, too, we should 
keep in mind, need not be particularly severe, nor need the 
initial general symptoms have been especially grave ; only the 
conjugate deviation of the eyes and head is a symptom which 
15 



226 DISEASES OF THE BRAIN PROPER. 

preferably occurs in grave hemiplegia. Its presence, therefore r 
permits a priori of an unfavorable prognosis with regard to 
complete recovery. 

In the first three or four weeks things remain apparently 
about the same ; the paralyzed side is flaccid and about five 
ninths to one degree centigrade warmer than its fellow, the 
slightest motion is impossible, speech remains impaired, and 
the face is one-sided. It is not until from three to six months 
have passed that we are able to notice a slight improvement in 
the power of motion, so that the patient (who is still confined 
to bed) is able to move with ease some of his toes, perhaps also 
the lower leg, while in the thigh motion is still incomplete, and 
in the arm and hand quite impossible. In such cases all the 
improvement that can be expected is but small and the damage 
which the stroke leaves very apparent. After from six to 
twelve months the patient again begins to be able to use the 
paralyzed leg, which in the meantime, in consequence of the 
flaccid condition of the ankle, has become longer. The walk is 
then very characteristic. Flexion in the hip being insufficient, 
the affected leg is brought forward by the aid of the pelvis, so 
that, trailing along the ground, it describes a half circle around 
the sound one. The centre of gravity of the body then is 
transferred to the paretic leg, the knee joint passively extended, 
and the leg thus used as a stilt (Wernicke). If improvement 
goes on, the movement ot circumduction gradually disappears 
and the paretic leg is simply dragged behind. The gait is so 
characteristic that the diagnosis, especially w T hen simultaneous- 
ly there is a paretic condition of the upper extremity, can be 
made at a glance. 

The upper arm is slightly abducted, the forearm flexed, the 
hand hangs down, the fingers, which are fixed in a somewhat 
flexed position, are completely useless, and the patient is un- 
able to grasp large or small objects. The arm can hardly be 
raised at all, and the movements of the forearm on the upper 
arm are very limited. In the lower leg extensor are more fre- 
quently developed than flexor contractures, and it is remark- 
able that in the morning, when the patient awakens after a long 
sleep, how slight they are and how little they trouble him, 
whereas in the course of the day they are materially increased. 

Contractures, which are in old hemiplegias hardly ever ab- 
sent, are most likely to be attributed to a shortening of the 
muscles produced by disuse. This idea is supported by the 



THE CEREBRAL HEMIPLEGIA. 



227 



fact that by systematic passive exercise, begun as soon as pos- 
sible, we are able to prevent contractures ; and if they exist, a 
proper galvanic treatment, which takes the place of passive 
motion, perceptibly diminishes them. It is true it remains 
unexplained why contractures are not found in all cases, and 
why in some the paralyzed extremities remain for life flaccid. 
That anatomical changes, too, especially, as Charcot assumes, 
the secondary degeneration of the pyramidal tract, are not 
without influence, and that, at any rate, the contractures are 
more marked the farther this secondary degeneration advances, 
can not be denied. 

A symptom which accompanies contractures, but which 
often occurs much earlier, is an increase in the tendon reflexes 
on the paralyzed side. Tapping of the triceps and biceps 
tendon of the arm, of the patellar tendon, and the tendo 
Achillis evokes lively muscular contractions. From the last 
named — the tendo Achillis — we can also obtain the so-called 
ankle clonus, of which phenomenon we shall speak later. Even 
tapping of bones is attended by jerkings, which are best seen in 
the leg when the tibia is struck (" periosteal reflex "). Here 
again we must leave the question open whether this increase 
in the reflexes is due to the degeneration in the pyramidal 
tracts or merely connected with the suspension of certain re- 
flex-inhibiting influences in the brain. In favor of the latter 
hypothesis speaks the fact that this increase in the reflexes is 
occasionally observed as early as a few days after the stroke, 
at a time when there can be no question of degeneration in the 
spinal cord. 

With the skin reflexes it is just the reverse ; they are usual- 
ly entirely lost on the paralyzed side or are at least decidedly 
diminished. This is especially the case for the abdominal and 
cremasteric reflexes, which can only in exceptional cases be 
obtained on the affected side. 

Sensation either returns soon after the initial disturbance or 
is permanently lost. In the latter case — i. e., where besides the 
hemiplegia there exists also a hemianesthesia — the lesion is to 
be located in the posterior portion of the posterior limb of the 
internal capsule. The hemiansesthesia takes in, in pronounced 
cases, the whole half of the body, including the mucous mem- 
branes, and extends as far as the median line. Face and trunk 
are equally affected ; occasionally we may find that the tri- 
geminus remains exempt. 



228 DISEASES OF THE BRAIN PROPER. 

In slight cases the disturbance is confined to the extremities 
and concerns more the sensibility to touch than the sensibility 
to pain. The patient feels the prick of a pin, but is unable to 
direct his fingers properly if the eyes are closed ; he makes 
mistakes in recognizing objects which are given him to feel; 
he is unable to fasten small buttons, etc. Changes in the mus- 
cular sense also may exist for a considerable time, the patient 
being unable with his eyes closed to give any information 
about the position into which his hand has been brought. 

In examining sensation in hemiplegics, Oppenheim (cf. lit.) 
has noticed that at times bilateral impressions are appreciated 
only on one side ; that, for instance, if a patient is pricked 
simultaneously in the right and left thigh, he only perceives 
one prick — -namely, that on the well side. 

One of the rarest of sensory disturbances is the persist- 
ent hyperassthesia of the paralyzed side, described by M. H. 
Fischer (Arch, de phys. norm, et path., February 15, 1887, ix, 

P . 185). 

The psychical condition is not always the same. In certain 
cases the patients seem to have regained all their former facul- 
ties satisfactorily, so that a careful examination brings to light 
nothing more than a slight loss of will power and of the capa- 
city for grasping ideas ; but in other instances the patient be- 
comes mentally weaker and at the same time irritable. He is 
easily made to cry and is liable to sudden changes of temper. 
Such patients are, however, notwithstanding their apparent 
obstinacy, very manageable and easily guided. Again, there 
are cases in which the mental weakness becomes very /ap- 
parent. The patient forgets the commonest things, the num- 
ber and the names of his children, confuses things and places, 
does not know what day of the week and what season of the 
year it is, etc. ; at the same time he may have different de- 
lusions and hallucinations. Some cases finally go on to com- 
plete dementia, which takes a course not unlike that of general 
paralysis. Legrand du Saulle has published an interesting 
study of such disturbances among the apoplectics of the Salpe- 
triere (Gaz. des hop., 68-71, 1881). 

In the further course of severe hemiplegias where regen- 
eration is impossible to any great extent, motor disturbances 
which we have designated as posthemiplegic (cf. lit. under 
treatise of Greidenberg) may follow. One of these is the so- 
called hemichorea, consisting of involuntary irregular move- 



THE CEREBRAL HEMIPLEGIA. 



229 



ments in the paralyzed limbs, which become aggravated by 
every mental emotion and voluntary movement and which en- 
tirely cease during sleep. These movements, which are best 
studied on the upper extremity, occur more frequently after 
cerebral infantile hemiplegia than in any other affection. The 
" hemiataxia " described by Grasset (cf. lit.) is closely related 
to hemichorea, and ought to be regarded as a variety of it. 
According to Charcot, the seat of the lesion in these cases is in 
the posterior portion of the internal capsule, the posterior part 
of the optic thalamus, and in the foot of the corona radiata. 
The so-called hemiathetosis will be considered in the chapter 
on the cerebral palsies of children. 

A second class of motor disorders is made up of those pe- 
culiar involuntary movements which have been described as 
"associated movements." They are observed in the paralyzed 
extremity when the patient moves the corresponding, unaf- 
fected, one ; thus, for instance, if a patient uses his right, well 
arm, the paralyzed arm makes similar movements, of course 
being restricted to a lesser or greater extent by any contract- 
ures which may be present. These movements have nothing 
in common with the so-called reflex movements which are 
found to occur in the paralyzed limb on stimulation of the 
sound one by the prick of a pin, the faradic current, etc. A 
peculiar instance of " associated movements " in an old hemi- 
plegia I had the opportunity of observing for months. It was 
as follows : Every time the patient yawned the left arm was 
raised involuntarily at the shoulder-joint, and was kept up 
while the yawning continued ; as soon as it ceased the arm 
dropped down helplessly. Sometimes one sees the sound 
limbs make involuntary movements if the patient attempts to 
use the affected ones, and again and again I have seen patients, 
straining to bend the paralyzed leg, become greatly astonished 
at the flexion which took place in the well leg without any 
such intention on their part. That in intended movements of 
certain muscle groups the antagonists begin to make involun- 
tary movements — that, for instance, if an extension of the flexed 
fingers be attempted, the flexion at first becomes more forcible 
before extension begins (Hitzig) — is, according to our experi- 
ence, very exceptional. 

There are other associated movements which occur in the 
paralyzed half of the face when the sound side is moved ; thus, 
for instance, in laughing, the muscles of the paralyzed side are 



23O DISEASES OF THE BRAIN PROPER. 

seen to contract equally, or even more strongly, than those of 
the well side. 

Various theories have been proposed to explain associated 
movements (Westphal, Benedikt, Broadbent, Ross), but none 
of them can be taken as entirely explaining the facts. It is 
by no means impossible that all such motor disturbances are 
reflex in nature (Charcot and Brissaud ; cf. also Senator, Ueber 
Mit- und Ersatzbewegungen bei Gelahmten, Berliner, klin. Wo- 
chenschr., 1892, 1). 

As a third posthemiplegic phenomenon we have the tremor. 
It is not rare, and that form especially which occurs on volun- 
tary movements of the affected side is rather frequently met 
with ; on the other hand, we shall very rarely have the oppor- 
tunity of observing this tremor while the extremities are at 
perfect rest. Relatively, the largest number of cases who 
presented tremor, in my experience, showed sensory changes, 
which consisted of paroxysms of pain in the affected extremi- 
ties. On a cursory examination this tremor may be mistaken 
for unilateral paralysis agitans (hemiparalysis agitans), espe- 
cially as the number of oscillations is about the same in both 
affections, 4% to sV 4 m a second. Pronounced intentional 
tremor, which we look upon as a pathognomonic symptom in 
multiple sclerosis, I never have observed in hemiplegia. Prob- 
ably the cause of this posthemiplegic tremor has to be sought 
in the general increase of reflex activity, which, as we may 
remark here by the way, is observed besides only in a very 
few cases of tremor of a different nature. Here it seems to 
play the most important role. 

Of great interest, as well as, at times, of no small practical 
importance, is the fact that in cases of incurable hemiplegia 
the non-paralyzed side, that is, the apparently well extremities, 
undergo certain changes which we are compelled to regard 
as pathological. Thus, Pitres has found that the well arm 
loses somewhat in strength, and that this is often more marked 
in the beginning of the hemiplegia than later on. On an aver- 
age the loss amounted to about 38 or 40 per cent, while no in- 
crease in the tendon reflexes could be demonstrated at the 
same time. The well leg becomes weaker, and indeed in a 
more marked degree than the arm, the strength being reduced 
in some cases even by one half. The patient, though able to 
move that leg with perfect ease while in bed, finds it almost 
useless to attempt to stand or walk. Pitres was also the first 



THE CEREBRAL HEMIPLEGIA. 23 1 

to notice that the patellar reflex of the sound side, as well, is 
abnormally active, an observation which is daily confirmed. 
The presence of the ankle clonus is noted by Westphal and 
Dejerine. All authors, however (Hallopeau, Brissaud, Fere), 
agree that it is extremely unusual to find the later contrac- 
tures on the non-paralyzed side. On the whole, these changes, 
which occur on the so-called unaffected side, are more marked 
and of greater significance to the patient than we should be led 
to suppose from a superficial examination. 

LITERATURE. 

Maeltzer. Ueber Pseudo-Apoplexien im Gehirn. Inaug. -Dissert., Breslau, 

1 881. 
Loewenfeld. Studien iiber Aetiologie und Pathogenese der spontanen Hirnblut- 

ungen. Wiesbaden, 1886. 
Hochhaus. Berliner klin. Wochenschr., 1887, 1. (Meningitic Hemiplegia.) 
Loewenfeld. Zur Lehre von den Miliaraneurismen des Hirns. Wiener med. 

Wochenschr., 1887, 47. 

Greidenberg. Arch. f. Psych., 1886, xvii, 1. (Post-hemiplegic Motor Disturb- 
ances.) 

Stephan. Revue de med., 1887, 3. (Pre- and Post-hemiplegic Tremor.) 

Brissaud et Marie. Progres med., 1887, 5, 7. (The Condition of the Facial in 
Hysterical Hemiplegia.) 

Wernicke. Berliner klin. Wochenschr., 1889, 45. 

Schaffer. Beitrag zur Lehre von der secundaren und multiplen Degeneration. 
Virch. Arch., 1890, cxxii, 1, 2. 

Scheiber. Arch. f. Psych., 1890, xxii, 1, p. 221. ("Athetosis spastica.") 

Erben. Versuch einer Erklarung fiir das verschiedene Verhalten der Sehnen- 
reflexe nach Hirnblutungen. Wiener med. Wochenschrift, 1890, 45. 

Strauss. Zweihundert und funfzig Falle von rechts- und links-seitiger Hemi- 
plegie ; ein Beitrag zur Frage nach der functionellen Verschiedenheit beider 
Hemispharen. Inaug.-Dissert., Berlin, 1890. 

Vizioli. Gaz. degli ospedali, 1892. 

Trophic vaso-motor changes are not uncommon in the par- 
alyzed limbs. While in the beginning of a hemiplegia the 
skin of the affected side is warmer and redder than that of the 
well side, it becomes cooler as the disease progresses, and fre- 
quently assumes a somewhat cyanotic color. The oedema 
often seen in the affected extremities is due to the absence of 
muscular movement and the consequent slowing of the blood 
and lymph current. In a patient who, two years before, had a 
pretty severe apoplectic attack with persistent speech disturb- 
ance, I have repeatedly observed slight repetitions of the 
haemorrhage, during which the speech, which had consider- 



232 



DISEASES OE THE BRAIN PROPER. 



ably improved, again became entirely unintelligible. Simul- 
taneously there was developed on each such occasion over the 
whole body, and not merely over the paralyzed right side, an 
urticarial rash which persisted as long as the cerebral symp- 
toms lasted. No doubt this was due to a disturbance in the 
vaso-motor innervation of the vessels of the skin, which reap- 
peared with the transient increase in the intracranial pressure. 
Charcot describes an acute malignant bed-sore which appears 
two or three days after the onset of the hemiplegia in the 
gluteal region, beginning as a red spot and developing in a few 
days into a brown, dry eschar six to seven centimetres broad. 
It always ends fatally, and is, according to Charcot, a purely 
trophic disturbance, an alteration in the tissue, which we can 
attribute only to nervous influences. 

The nutrition of the muscles which for years have been par- 
alyzed usually suffers but little. We can easily understand 
that a slight degree of atrophy, due to inactivity, occasionally 
manifests itself, yet the excitability to both electrical currents 
remains normal. Only in exceptional cases is there pronounced 
muscular atrophy in the affected limbs when these, although 
their motion is impaired, can still be used to a certain extent. 
In such cases the atrophy can not be referred to inactivity, but 
we must rather assume a lesion in the trophic centres of the 
cortex, the seat of which is, however, still unknown. Since 
these conditions have received considerable attention of late, 
we add here some references. 

LITERATURE. 

Quincke. Deutsches Arch. f. klin. Med., 1888, xlii, 5. 

Borgherini. Ueber die friihzeitige Muskelatrophie bei der cerebralen Lahmung. 

Deutsches Arch. f. klin. Med., November, 1889, xlv, 5, 6. (Atrophy on 

the Third Day after Hemiplegia; the lesion was thought to be in the 

thalamus.) 
Eisenlohr. Muskelatrophie und electrische Erregbarkeitsveranderungen bei 

Hirnherden. Neur. Centralbl., 1890, 1. 
Darkschewitsch. Zwei Falle von friihzeitiger Muskelatrophie bei einem Hemi- 

plegiker. Neurol. Centralbl., 1891, 20. (D. regards it as a cerebral 

amyotrophy.) 
Mouratoff. Arch, de Neur., 1891, Ixiii, p. 461. 
Steiner. Ueber die Muskelatrophie bei der cerebralen Hemiplegia Deutsche 

Zeitschr. f. Nervenhk., 1893, iii, 4, 5. 

The simultaneous appearance of a haemorrhage in each 
hemisphere is exceptional. It needs hardly to be stated that 



DIAGNOSIS OF CEREBRAI HEMORRHAGE. 



233 



such an accident must necessarily give rise to the gravest 
symptoms : bilateral hemiplegia — that is, paralysis of all four 
extremities — bilateral facial and hypoglossal paralysis, amauro- 
sis and total anaesthesia. 

Diagnosis. — The diagnosis of cerebral haemorrhage may 
give rise to considerable difficulties. It is easy only when a 
suddenly or gradually developing unconsciousness is followed 
by a paralysis or paresis of one side in a patient not suffering 
from any valvular disease of the heart. Under such circum- 
stances the case is absolutely clear, and even the most cautious 
diagnostician, if he can exclude hysteria, may safely assume a 
cerebral haemorrhage with consequent hemiplegia. 

It is a different matter where we have to make a diagnosis 
at a time when we are unable to ascertain the presence or 
extent of the paralysis, but where we are restricted to an in- 
terpretation of the unconsciousness of the patient. Under 
these circumstances w r e have to be familiar with the conditions 
which, besides cerebral haemorrhage, are capable of giving rise 
to unconsciousness, and be acquainted with the characteristic 
manifestations which each offers. 

In the first place we may have to deal with a simple faint- 
ing fit. The concomitant symptoms — the wax-like pallor of 
the face, the small, frequent pulse, the cold sweat which covers 
face and body — are not likely to allow us to mistake the con- 
dition for one of apoplexy, especially as the gravest symptom 
— the loss of consciousness — as a rule, is not of long duration, 
but vanishes rapidly if the patient is laid down with the head 
low, the face sprinkled with cold water, or if ammonia or eau 
de Cologne, etc., be held to the nose. The success or non- 
success of these measures will help us to settle the differential 
diagnosis in a few minutes. 

Secondly, we may have before us an epileptiform attack 
without convulsions or the coma which so often follows epilep- 
tic fits. Here the loss of consciousness is also complete, and 
the diagnosis can only be made if we can obtain a history of 
previous epileptic convulsions, or if we are able to assume this 
from scars on the tongue. In the absence of such evidence 
the color of the face may sometimes be of value to us ; in some 
epileptics this is very pale, in cerebral haemorrhage of a pur- 
plish color, yet this rule by no means always holds good, and 
should therefore be accepted cum grano salis. 



234 DISEASES OF THE BRAIN PROPER. 

The unconsciousness so often occurring in the course of 
a meningitis may be recognized from the temperature and the 
pulse, the peculiar drawing in of the abdomen (scaphoid abdo- 
men), the jactitations, the rigidity of the neck, and possibly 
from the existence of choked disks. The possibility of an 
internal pachymeningitis hsemorrhagica must be thought of 
when the development of the condition has been characterized 
by sudden exacerbations and remissions, and when at the same 
time a history of alcoholism can be obtained. 

In the beginning and in the course of progressive paraly- 
sis of the insane (dementia paralytica) apoplectiform attacks 
occur which resemble those produced by cerebral haemorrhage 
very closely indeed, and which can be recognized as belonging 
to the former disease only from the previous history of the 
patient (and later from the results of the autopsy). If we can 
get no information from the history the differential diagnosis 
is impossible. 

Intoxication with chloroform and alcohol may be attended 
by complete loss of consciousness. An individual in the uncon- 
sciousness of alcoholic intoxication is just as hard to arouse as 
one in apoplectic or epileptic coma, and the diagnosis may pre- 
sent some difficulties under certain circumstances — when, for 
instance, nothing can be learned about the cause, or what has 
immediately preceded the loss of consciousness. Usually, how- 
ever, it is easy enough. Sometimes the smell of the ingested 
substance puts us on the right track, sometimes prompt reac- 
tion to energetic stimuli applied to the skin may make our 
diagnosis clear. As long as we are not sure of our ground, we 
ought to abstain from all therapeutic measures. Of opium or 
of morphine poisoning we need only think when the pupils of 
the patient are conspicuously small. A degree of myosis as 
high as we find in opium poisoning has only its parallel, and 
then but rarely, in haemorrhages into the pons, which are 
rapidly fatal. 

Urasmic coma can easily be excluded, if we are able to ex- 
amine the urine, and can detect neither albumin nor tube casts ; 
besides this, with the history, the examination of the heart for 
a possible hypertrophy should not be forgotten. 

Diabetic coma, finally, is characterized by a peculiar fruity 
odor which comes from the mouth of the patient. It, of course, 
only enters into the question if sugar can be demonstrated (or 
has previously been repeatedly detected) in the urine. 



DIAGNOSIS OF CEREBRAI HEMORRHAGE. 



235 



It is not common to encounter any difficulty in deciding 
which side is paralyzed ; nevertheless I have seen instances in 
which this was the case. Thus it occasionally happens that, 
owing to the deep coma in which the patient lies, the limbs of 
both sides fall equally flaccidly when allowed to drop, while no 
difference can be discovered in the two sides of the face. In 
such cases it is well to throw some ice-water over the patient, 
upon which it will be observed that he will make movements 
of defense only with the non-paralyzed side, and the facial 
muscles will contract only on that side. 

The anatomical nature of the hemiplegia may remain en- 
tirely obscure, and only in certain cases are we able to give a 
decided opinion about it. 

Whether hemiplegia following a stroke is due to haemor- 
rhage or embolism can only be determined by accompanying 
circumstances. The existence of valvular lesions and of athe- 
roma speaks for embolism ; nephritis, heart hypertrophy, albu- 
minuria, for haemorrhage ; yet this rule has many exceptions, 
and we may assume that in about half the cases a correct diag- 
nosis is impossible (cf. Dana, Med. Record, 1891, p. 30). 

The meningitic hemiplegia has these points in common 
with the hsemorrhagic — namely, the paralysis on one side and 
the " conjugate deviation " ; but, as we have before pointed out, 
in meningitis we generally have the characteristic rigidity of 
the neck and the scaphoid abdomen ; where these latter symp- 
toms are not even suggested, a differential diagnosis, or rather 
the recognition of a hemiplegia as of meningitic origin, is im- 
possible. 

The hysterical hemiplegia, finally, if it persist for a long 
time, and if other hysterical symptoms, as anaesthesias or con- 
tractures, are wanting, can never with any certainty be differen- 
tiated from that depending upon cerebral haemorrhage. Both 
may present the same peculiarities, and a decision as to which 
condition we are dealing with may be beyond the powers even 
of the practiced diagnostician. We are indebted to Charcot 
for a new symptom, to which he has drawn attention, and 
which is said to be characteristic of hysterical hemiplegia — 
namely, a paroxysmal spasm of the muscles of the cheek of one 
side, associated with an excessive deviation of the tongue to 
the same side. This " glosso-labial hemispasm " never exists in 
organic lesions of the pyramidal tract, and is therefore pathog- 
nomonic for hysterical hemiplegia (Brissaud and Marie, cf. lit.). 



236 DISEASES OF THE BRAIN PROPER. 

If the question of the anatomical seat of the haemorrhage is 
to be considered in our diagnosis, we must in the first place 
not forget that the mere existence of a hemiplegia is not suf- 
ficient to give us an answer, for as long as we do not know 
whether to regard it as a direct or indirect symptom, we 
can say nothing positive. If we further add that even an indi- 
rect hemiplegia may persist for years, we can easily see with 
what difficulties we meet in attempting a topical diagnosis. It 
may be quite true that in a great many cases where an apo- 
plectic attack is followed by hemiplegia, the lesion is situated 
in the internal capsule, and we have become accustomed to as- 
sociate in our minds a certain typical clinical picture — that is, 
hemiplegia with more or less marked sensory changes — with a 
lesion in the internal capsule. We must, however, in making a 
diagnosis of that kind, always keep in mind that an indirect 
hemiplegia may be produced by lesions in any part of the 
brain, by lesions in the frontal, in the parietal, the occipital 
lobe, of the thalamus, of the lenticular nucleus, of the external 
capsule, and that, as we have also said, the duration of such in- 
direct hemiplegias is by no means always restricted to a period 
either of a few days or a few weeks. Hence a certain reser- 
vation must ever be observed by a prudent diagnostician, and 
he should speak with some certainty only when he has some 
other direct focal symptom to guide him. Among these, we 
have, for instance, sensory aphasia for the (left) temporal lobe ; 
for the occipital lobe, hemianopia ; for the optic thalamus (with 
a high degree of probability), posthemiplegic chorea ; for the 
crura, alternating oculo-motor paralysis ; for the pons, alternat- 
ing facial paralysis. According to Durck, it is possible at au- 
topsy to determine approximately the age of the haemorrhage 
from the condition of the red corpuscles (whether they are 
normal, discolored, swollen, shrunken, etc.), and from the ana- 
tomical and chemical condition of the blood pigment. If these 
points are taken into consideration, its age within a period of 
from one to seventy-two days may be estimated (cf. Virch. 
Arch., 1892, cxxx, Heft 1, p. 89). 

Prognosis. — After all that has been said, we hardly need to 
add anything about the prognosis. Any cerebral haemorrhage 
is a grave event, which puts the life of the patient in danger, or 
rather it is a symptom which denotes that a grave arterial dis- 
ease, without which a haemorrhage never occurs, has reached 
a state dangerous to life. If once a haemorrhage has occurred 



PROGNOSIS IN CEREBRAL HAEMORRHAGE. 



237 



we are not sure but that it may be repeated at any moment, 
since the condition which favored it, the brittleness of the ar- 
terial walls, means a lasting incurable predisposition to a fresh 
haemorrhage. 

In the presence of a recent apoplectic attack, it is impossi- 
ble for us to give a certain prognosis, or to predict what will 
follow. The severity of the disturbance of consciousness is in 
a way indicative, and we may say that the severer this is 
found to be — in other words, the greater the traumatic effect of 
the haemorrhage — the less favorable is, cceteris paribus, the out- 
look with regard to life, as well as with regard to recovery. 
Yet exceptions occur, and even a very severe coma which has 
persisted for hours does not only not always produce death, 
but need not necessarily leave behind it focal symptoms, as 
hemiplegia or the like, and such patients may then be well for 
years afterward. Unfortunately, so favorable a result is rarely 
met with. As a rule, a haemorrhage of any considerable size 
is either fatal or is followed by a hemiplegia. 

As to the difference in the prognosis for the individual, in- 
direct as well as direct focal symptoms, most that deserves 
mention has already been spoken of. The indirect symptoms, 
as a rule, disappear after a certain time, and a restitutio in in- 
tegrum is not impossible ; the direct ones are only curable 
when vicarious innervation takes place from the unaffected 
hemisphere which assumes the function of the damaged one. 
This can be the case, for instance, in unilateral facial and hypo- 
glossal paralysis, and in the lateral deviation of the eyes (lesion 
of the lower parietal lobule) ; it may also occur in motor 
aphasia if the patient is still capable of learning to speak with 
his right hemisphere (lesion of the region of Broca). On the 
other hand, it does not occur in cases of direct hemiplegia due 
to a lesion of the internal capsule ; then the paralysis is incur- 
able, and the improvement which may take place is always 
very imperfect, although a properly conducted treatment may 
effect some amelioration, and thus conduce much to the well- 
being of the patient. 

Treatment. — The primary affection, the disease of the ar- 
teries to which cerebral haemorrhage is due, is beyond the 
reach of therapeutics. We possess no remedy which can 
cause the miliary aneurisms to disappear, and our efforts are 
confined to combating those symptoms which accompany and 
those which follow the haemorrhage. Thus we have to deal 



238 DISEASES OF THE BRAIN PROPER. 

with (1) the apoplectic attack itself ; (2) the anatomical changes 
which are produced in the brain by the haemorrhage ; (3) the 
focal symptoms, the paralysis (or paresis) of one side ; and, in 
general, all motor and sensory disturbances referable to the 
attack. 

(a) The treatment of the attack itself varies according as we 
have to deal with a suddenly or gradually developing apo- 
plexy. In the former case we may assume that the haemor- 
rhage has already stopped when we first see the patient, 
whereas in the second case the presumption that the bleeding 
is still going on is justifiable, and hence all measures which 
tend to arrest the haemorrhage are strenuously indicated at 
once. One of these is venesection, which produces a fall in 
the blood pressure, and should always be resorted to in cases 
in which, after (or during or perhaps before) a haemorrhage, the 
carotids are found throbbing, the action of the heart is tumul- 
tuous, and the face red and congested. The success is some- 
times surprising. The patient, who just before was comatose 
and motionless, with stertorous breathing, immediately after a 
free bleeding begins to breathe more quietly, and evidently 
with greater ease. He stirs, opens his eyes, and becomes con- 
scious. In such a case venesection was the only measure indi- 
cated ; it could not have been replaced by anything else — in 
short, it has saved the patient's life. The compression of the 
carotid artery, which Spencer and Horsley recommend as a 
result of their experiments upon animals, will probably be re- 
sorted to only in rare instances (Brit. Med. Journ., March 2, 
1889). If the pulse is small, the face pale, and the heart sounds 
are weak, no one will ever think of taking away blood. Then 
the administration of stimulants will be found useful; of course, 
they have to be given with great caution, and be selected care- 
fully. Vinegar enemata, sinapisms, and ether injections may 
be tried. Changes in the blood pressure of the brain ought to 
be avoided most carefully ; they may be produced by turning 
the patient in bed, by shouting at him frequently, and by other 
attempts to wake him from his coma. The physician will have 
to warn the friends against doing this, and do his best to have 
the patient left quiet and undisturbed. If the face is congested, 
he will order his head to be placed high and have him kept in 
one position. Local bleeding from the head is, if not directly 
harmful, absolutely useless. If bleeding is indicated at all, we 
shall choose venesection ; cupping and leeching are matters of 



TREATMENT OF CEREBRAL HEMORRHAGE. 



239 



so much detail and are so slow in their action that they can not 
be recommended. 

Immediately after the attack has passed off and the patient 
has regained consciousness the chief task of the physician is to 
see that he has absolute rest. Even more than any other sud- 
den illness, apoplexy produces the greatest excitement and 
consternation in a family, and it can hardly be wondered at 
that this gives place to the greatest joy when the patient, who 
has already been given up, is seen to return to life, and that 
each member of the family is anxious to express his feeling of 
satisfaction. All such outbursts may be very harmful to the pa- 
tient, and these demonstrations must be crushed by the physi- 
cian with iron firmness in order to avoid any emotion on the 
part of the patient ; besides, he should give directions as to a 
proper bed which will answer all the therapeutic and hygienic 
requirements of the case, and, above all, from the very first 
due precautions against bed-sores ought to be taken. Proper 
arrangements should be made for the reception of the stools 
and the urine. The head ought to be covered with thin com- 
presses, cooled with ice-water or with a light ice-bag that will 
exert no pressure. The application of cold must not, however, 
be carried too far, since by a contraction of the peripheral 
vessels we run a risk of producing an increase in the intra- 
cranial blood pressure, which would be the opposite of what 
we are attempting to do. Any simple medicine which con- 
tains acids or cream of tartar or tartar, boraxat. and the like, 
is sufficient for the first few days, during which the patient 
ought to be fed upon a light, nourishing, but unstimulating 
diet. 

(b) The treatment of the focal lesion in the brain — that is, 
of the place where the haemorrhage has occurred — should not 
be begun until a considerable time has elapsed after the gen- 
eral symptoms have abated. This will usually be from about 
four to six- weeks after the attack. Whether it is actually 
necessary to wait so long we do not know, but, as a matter of 
fact, we are afraid to undertake any active measures at an 
earlier moment, and certainly if a physician should go contrary 
to tradition, and if accidentally another haemorrhage should 
occur, he would lay himself open to severe censure on the part 
of the family. 

On the other hand, it seems more than doubtful whether 
we are able to influence the disease-focus in any way by treat- 



240 DISEASES OF THE BRAIN PROPER. 

ment or succeed in hastening- the absorption which we desire. 
It is, however, supposed that this can be accomplished in two 
ways : namely, by internal and external remedies, by potassium 
iodide and mercury, and by galvanic treatment respectively. 
The iodide treatment is based on the supposed absorbent prop- 
erties of the drug. Whether it possesses such a power to any 
great degree is doubtful, and the fact that it so frequently fails 
to give good results seems to speak very much against it. On 
the other hand, there is no question but that iodide, if used for 
any long period of time, acts deleteriously on the stomach, and 
spoils the appetite and may lead to symptoms of intoxication. 
An unprejudiced practitioner who does not administer medi- 
cine in a routine way will therefore always first ask himself 
which of the two is the lesser evil — whether he should renounce 
such help as is supposed to be derived from the remedy in the 
process of absorption and keep the patient's appetite in a good 
condition, or whether he should depend upon the more than 
doubtful action of the drug and at the same time ruin the pa- 
tient's digestion. But if we have once decided to administer 
iodide of potassium, let it be done boldly, and let 2.0, 3.0, 5.0 
(30, 45 to 75 grains) a day in one or two doses be given in hot 
milk. Given in this way the administration of the drug is less 
likely to be followed by unpleasant effects than if we order a 
tablespoonful three times a day of a solution of iodide, 4.0 to 8.0 ; 
water, 200 ( 3 j - 3 ij to 3 vj). The mercurial inunctions to the 
portion of the skull corresponding to the focus are not harmful 
if any symptoms of intoxication are watched for and salivation 
is at once treated energetically ; but their success is in no way 
greater than that obtained with potassium iodide. 

With regard to the galvanic treatment, it must first of all 
be absolutely admitted that it is possible to act upon the brain 
with the galvanic current. The peculiar phenomena which 
occur during galvanization of the head — vertigo, seeing of 
sparks, etc., the cerebral nature of which can not be doubted — 
speak strongly in favor of such a possibility, and the experi- 
ments of Loewenfeld on animals seem to indicate that these 
are due to an influence on the circulation in the brain. Whether, 
however, the galvanic current possesses, besides this undoubted 
action on the vaso-motor nerves, definite catalytic properties, 
and, if so, in a measure sufficient to enable us with its help to 
influence the disease-focus, nobody knows. We will suppose 
this and hope that it is so, because it is the only weapon upon 



TREATMENT OF CEREBRAL HEMIPLEGIA. 



2 4 1 



Avhich we have to depend. The best and most reliable electro- 
therapeutists, Erb at their head, with his unusually wide ex- 
perience, admit the scarcity of positive results from such a 
treatment, and acknowledge that in by far the greater number 
of cases they are negative (Erb, Handbuch der Electrothera- 
pie, page 320, Leipzig, 1882). Yet cases may occur where the 
physician is forced to resort to galvanization of the head — 
" the electrical treatment of the brain." He should therefore 
be familiar with the mode of application. Only large electrodes 
ought to be used. Fig. 73 represents the head electrode of 
Erb. The anode being placed on the forehead, the cathode on 
the neck, weak currents without make or break should be al- 




Fig. 73.— The Large Head Electrode (covered with Sponge) of Erb. 

lowed to pass through the head of the patient for from one 
minute and a half to two minutes. Transverse and oblique 
currents may also be tried. (Details are to be found in Hirt, 
Lehrbuch, be. cit., page 165.) 

(e) The treatment of the hemiplegia and the posthemiplegic 
motor disturbances demands, if any success is to be expected, 
much perseverance on the part of the patient as well as of the 
physician. If we can not familiarize ourselves with the idea 
that for weeks and months the same procedures and manipu- 
lations have to be gone through in exactly the same way, we 
should not begin the treatment at all nor order it to be under- 
taken. We shall then at least spare ourselves the disappoint- 
ment of a failure ; yet with patience, and where the necessary 
means are not wanting, it should be undertaken. The cases 
in which a systematic treatment for a long time has benefited 

the patient very materially are numerous enough, and thev 
16 



242 DISEASES OF THE BRAIN PROPER. 

would undoubtedly be met with more frequently if a fair trial 
were given it more often than is unfortunately the case. Gross- 
raann has shown that suggestion plays a prominent part in the 
results of this treatment (Die Erfolge des Suggestionstherapie bei 
nicht-hysterischen Lahmungen, Berlin, 1892). Since there can 
be no question of regenerating destroyed brain tissue, his aim 
is to produce an improvement in the general cerebral energy. 
To this point we shall return later. 

From internal medicines absolutely no effect on the hemi- 
plegia is to be expected, and even the most sanguine thera- 
peutists, whose faith in drugs is unbounded, abstain here from 
fruitless attempts. The same may be said about the posthemi- 
plegic motor disturbances, and if we here make mention of the 
use of veratrine (0.002 to 0.003 [gr. J / 30 to y 20 ] a day in pills) 
against the posthemiplegic tremor, it is only to declare the utter 
futility of this medicament. We have here also to resort chiefly 
to electrical treatment, but with this difference, that the good 
results observed are much more frequent and much more 
marked than in treating the disease-focus. Definite rules for 
the electrical treatment of the focal lesions as well as the hemi- 
plegic and the posthemiplegic phenomena can not be given. 
Every experienced electrotherapeutist follows certain rules 
and principles which he has found out for himself in the course 
of years from personal observation. Thus one claims only to 
accomplish his end with quite weak, while the other has seen 
better results from the use of strong currents. The one uses 
galvanism, the other by preference the faradic current. Every 
one adduces reasons for his own method, which, as a rule, are 
strongly combated by other writers who claim to possess in- 
finitely more experience. 

Above all, the wishes of the patient should guide us in de- 
ciding which mode of electrization should be chosen. One 
man will have a genuine idiosyncrasy against the faradic cur- 
rent, and more especially against the faradic brush. Another 
can not stand strong galvanic currents ; they excite him, make 
him nervous, and disturb his sleep. In a very general way we 
may lay down the rule that in paralytic conditions most is 
accomplished by the galvanic current, with frequent makes 
and breaks, so as to produce contractions of the muscles. In 
conditions of irritation, especially contractures, on the other 
hand, most is accomplished by local faradization. We hardly 
need to insist that the greatest attention must be given to the 



TREATMENT OE CEREBRAL HEMIPLEGIA. 



243 



groups of muscles most severely affected — for instance, in the 
upper extremities, to the extensors. The faradic treatment 
may, especially if contractures are threatening, be begun 
earlier than is allowed by our rule given above, even twelve 
to fourteen days after the cessation of the general symptoms, 
without any danger to the patient. 

Patients in good circumstances expect their physician to 
send them to a watering place every year, as a stay there is a 
pleasant change from the monotonous electrical treatment, and 
we can not blame anybody for putting great faith in it. Un- 
fortunately, these hopes are not by any means justified, and 
by a course of treatment at Oeynhausen, Wildbad, Gastein, 
and Ragatz, where, by the way, the temperature of the baths 
ought not to exceed 93 F., painfully little is accomplished, cer- 
tainly a great deal less than by electrization or this alternated 
with massage. The latter ought to be carried out only by 
well-trained masseurs, and only with the greatest care. From 
the cold-water treatment we also have seen little success on the 
whole, although it is decidedly to be preferred to the simple 
hot baths and the like. This also must be administered care- 
fully, and must be adapted to the idiosyncrasies of the patient, 
a rule which is unfortunately not always observed. Hydro- 
therapeutics can not be learned in the clinics, where only an 
occasional remark is made about it, but deserves and demands 
a practical study in establishments where this treatment is in- 
telligently and carefully conducted. The reason why it is not 
esteemed everywhere as highly as it ought to be is because it 
is frequently not understood. Those who wish to acquire the 
theory of this treatment thoroughly I would refer, among 
other works, to the excellent text-book of Winternitz. 

While we have seen, then, how helpless therapeutics is 
against cerebral haemorrhage and its consequences, we have, 
on the other hand, the satisfaction of knowing that so much 
success is promised by a timely and appropriate prophylaxis, 
that we must recommend it most earnestly to all individuals of 
a so-called apoplectic habit, all who are inclined to cerebral 
congestion, all patients with a heart hypertrophy, and, finally, 
all those with hereditary tendencies. They should try to avoid 
putting on too much flesh, and shun everything which would 
conduce to the production of an undue increase in the blood 
pressure. Among the most important rules upon which we 
must insist are moderation in eating, regulation of the bowels, 



244 DISEASES OF THE BRAIN PROPER. 

frequent exercise in the open air, systematic gymnastics in- 
doors — for instance, on the " ergostat " of Dr. Gartner, of 
Vienna, a small apparatus which can easily be kept in the 
room and on which a large amount of work, measured by 
kilogramme-metres, can be done (the work can be prescribed 
in kilogramme-metres). This apparatus I can highly recom- 
mend, as I have very often seen good results from its use. To 
avoid increase in the blood pressure, the use of alcohol, coffee, 
and other stimulants, finally, all excitement, be it sexual or of 
any other kind, should be interdicted. Unfortunately, these 
warnings of the physician are not listened to until it is already 
too late, and men who will protect themselves in time and give 
up some pet habit — the customary nap after dinner, or the like 
— in order to avoid a danger that only threatens, are few and 
far between. 

2. Embolism and Thrombosis of the Cerebral Arteries. 
Encephalomalacia. 

Pathological Anatomy. — We have already adverted to the 
fact that the arteries of the cortex anastomose among them- 
selves, while those of the basal ganglia are what we call ter- 
minal arteries ; from this it is evident that the embolus has 
quite a different significance where it plugs up an artery of 
the former type to that which it possesses when the ob- 
structed vessel is a terminal artery, and no collateral circula- 
tion is possible. In the first case the collateral circulation 
compensates for the damage, while in the second case we are 
bound to have a necrosis in the areas supplied by the ob- 
structed artery, a " focus of softening." It is unnecessary to 
dwell much upon the important bearing of this fact ; suffice 
it to say that the arteries usually concerned are the main 
branches and, above all, the middle cerebral. The reason 
why embolic processes are more frequent on the left than on 
the right side has already been explained. Brain emboli origi- 
nate in the same manner as emboli in other organs ; among 
the causes are diseases of the left heart — chronic endocarditis, 
mitral disease, and weak heart — aortic aneurisms, more rarely 
diseases in the pulmonary circulation. Thus in certain cases 
purulent particles may pass from the lungs into the pulmonary 
vein (in ulcerative bronchitis, gangrene of the lungs, etc.), and 
be carried into the systemic circulation. In a case reported 
by Dahnhardt a doubled-up echinococcus vesicle was the cause 



EMBOLISM OF THE CEREBRAL ARTERIES. 



245 



of embolism in the left art. fossas Sylvii, the left art. prof, 
cerebri, and the arter. basilaris (Neurol. Centralbl., 1890, No. 19). 
Polchen (cf. lit.) has also shown that certain poisons, espe- 
cially carbon monoxide, appear to sometimes produce soften- 
ing of the brain substance. According to him, the CO while 
circulating in the blood acts injuriously on the nutrition of 
the vessels, and brings about fatty degeneration and calcifica- 
tion in them. Hence there finally results a necrosis of the 
tissue. It is possible that phosphorus acts in a similar way. 
Age plays a still less important role in the setiology of embol- 
ism than in that of haemorrhage, whereas the influence of sex 
can not be denied, as it is well known that by far more women 
are attacked by cerebral embolism than men ; it is possible that 
this is the case, owing to the greater frequency with which we 
find articular rheumatism with its accompanying heart lesions 
in the female sex, especially in its younger members. The 
puerperal state may also have something to do with it. 

Thrombosis of the cerebral arteries is either produced by 
an atheromatous process which narrows the lumen of the ves- 
sel, and by slowing the blood current gives rise to coagulation, 
or by an abnormal proneness of the blood to coagulate. The 
first happens frequently in old people, and we can fairly say 
that atheroma is just as often the cause of senile softening as 
miliary aneurisms are the cause of cerebral haemorrhage. The 
abnormal tendency to coagulate (hyperinosis), which the blood 
presents in the puerperal state, in pneumonia, etc., is rarely or 
never the only cause of coagulation. It can not be said to do 
more than favor it, and hence we need not go further into the 
question. Considerable general increase in the intracranial 
pressure may give rise to thrombosis (compression thrombosis), 
as also the pressure exerted on the vessels which occurs some- 
times in basilar meningitis. If, in addition, the arterial walls are 
diseased— for instance, by tuberculosis or syphilis — the con- 
ditions are still more favorable for the formation of thrombosis. 
According to Gerhardt, the hemiplegias which occur in the 
course of basilar meningitis are due to thrombosis with second- 
ary softening. 

Finally, we should remember that traumatism — a fall or a 
blow upon the head— may produce a disease in the arteries 
which long after may give rise to thrombosis. 

The necrosis of the brain tissue which follows the cutting 
off of the arterial blood supply is called softening, encephalo- 



246 DISEASES OF THE BRAIN PROPER. 

malacia, and we speak, according to the special ^etiological fac- 
tor, of a traumatic, an embolic or thrombotic, and an atheroma- 
tous (senile) softening. The process is as follows (Wernicke): 
The vessels in the area from which the blood supply is cut off 
collapse, the lymph spaces dilate and through aspiration be- 
come filled with cerebro-spinal fluid, so that the whole tissue 
appears soaked, and the recent focus of softening shows a de- 
cided increase in volume; the nerve fibres and nerve cells then 
become macerated in the fluid, and soon undergo destruction. 
With the microscope we detect varicosities of the nerve fibres, 
myelin drops, and the neuroglia and the connective tissue 
appear cedematous. If many red corpuscles are present, the 
coloring matter coming from them gives to the whole focus 
a yellowish tint ; such a discoloration is especially seen in the 
cortex {plaques jaunes, Charcot) ; the white matter which lies 
beneath is usually of a lighter tint. If then no sufficient col- 
lateral blood supply is established, which, as seems not impos- 
sible in a recent focus, might produce complete regeneration, 
there commences to develop in from thirty-six to forty-eight 
hours a fatty retrograde metamorphosis of the necrotic tissue. 
Polynuclear leucocytes emigrate from the dilated blood-vessels 
and invade the necrotic tissue ; they take up the fatty parti- 
cles, and some reach the blood current again through the 
lymph channels as compound granular corpuscles. The latter, 
which are invariably present in foci of softening more than two 
days old, on account of their infiltration with fat granules, are 
larger than the normal leucocytes. A part of them seem to 
undergo fatty degeneration, others seem to be transformed into 
myelin drops, especially in old foci. A quite gradual absorp- 
tion of the dead and disintegrated brain tissue takes place, and 
a so-called cyst is formed, which can not be distinguished from 
that following a brain haemorrhage; more rarely we find a 
cicatrix of connective tissue, which becomes as hard as car- 
tilage, and grates under the knife. Softenings, which from the 
onset take a chronic course, have frequently been found to 
form sclerotic cicatrices, so that the softening can eventually 
become a sclerosis (Wernicke). 

In softening of the cortex quite considerable areas may be- 
come deficient, which are partly replaced by serous fluid, 
partly by thickened pia. The convolutions, which sometimes 
remain, present a yellowish discoloration, appear atrophic, and 
are of a firm sclerotic consistence. 



CEREBRAL EMBOLISM. 



247 



Symptoms and Diagnosis. — Just as in haemorrhage, we 
may in embolism have symptoms which have to be regarded 
as premonitory of the regular attack. They resemble very 
closely those above described, and chiefly consist of vertigo, 
headache, an occasional feeling as of pins and needles in the 
limbs, etc. The headache may be especially prominent; it 
may persist for weeks with undiminished intensity, and then 
disappear, or be followed by a distinct deficiency in memory 
or beginning mental decline. 

The attack proper, which occurs at the moment the lumen 
of the vessel is completely obstructed by the embolus, may 
simulate the apoplectic attack so closely that it may be abso- 
lutely impossible to distinguish the one from the other. All 
the above-described differences in the nature and degree of 
disturbance of consciousness may be met with here also, and 
though it is true that at times the attack sets in with more vio- 
lent epileptiform convulsions, that the face is less congested 
and respiration less disturbed, these points are by no means 
sufficient for a differential diagnosis. It is supposed that com- 
plete loss of consciousness speaks more against embolism and 
for haemorrhage, and the early disappearance of the paralytic 
symptoms present point rather to embolism. Gerhardt con- 
siders (Berl. klin. Wochenschr., May 2 and 9, 1877) a well- 
pronounced aphasia to be in favor of embolism in doubtful 
cases. 

In embolism the attack is not evoked by an increase in the 
blood pressure, as in apoplexy, but by a " negative pressure." 
" Since the vessels lying to the peripheral side of the embolus 
suddenly collapse, and the blood contained in the capillaries 
flows into the veins owing to the vis a tergo exerted by the 
contraction of the vessels, a vacuum is suddenly formed in 
the tissue, and hence a negative blood pressure is produced " 
{Wernicke, loc. cit., p. 133). In its efforts to fill up the empty 
space, the brain parenchyma is bound to be subjected to a 
more or less considerable traction from all sides, which may 
sometimes lead to disintegration. If only a very small area is 
affected by the embolus, a regular attack may not take place 
and consciousness not be lost ; if it is very large, various in- 
direct symptoms may appear, and indeed even the non-affected 
hemisphere be implicated. But even after a severe stroke and 
after consciousness has been lost for quite a long time, a favor- 
able event is by no means impossible, because the tissue does 



248 DISEASES OF THE BRAIN PROPER. 

not necessarily disintegrate, as in haemorrhage, but an equaliza- 
tion of the blood pressure can take place, which will cause the 
disappearance of all the symptoms. 

Cerebral thrombosis rarely gives rise to a stroke, owing to 
the slowness with which the process takes place, and when an 
apoplectiform attack actually does occur, it must be due to the 
previous obstruction of other, neighboring, vessels. We had 
a considerable area dependent for its blood supply on a single 
vessel which before remained open, but has now gradually 
become so narrow that the pressure in it becomes too low to 
keep up the function (Wernicke). 

The necrosis (softening, encephalomalacia) to which the ob- 
struction of an artery, if lasting sufficiently long, is bound to 
give rise, manifests itself by certain focal symptoms, which 
may, just as in haemorrhage, be divided into direct and indi- 
rect. Among the indirect the hemiplegia, often attended with 
hemianaestbesia, which closely resembles that described above, 
is the most important. Monoplegias also and hemianopia may 
set in without a definite stroke, and may be produced indirectly 
from the focus of softening, which lies in close proximity to 
the part the functions of which are interfered with. If an em- 
bolus obstruct an artery which can communicate by anasto- 
moses with those of neighboring areas, and thus the damage 
can be compensated, we shall meet with transient focal symp- 
toms (Wernicke), which at the most require eight days for 
complete recovery. 

To determine the exact seat of the focus of softening, we 
must go to work with the same caution as in making a topical 
diagnosis of a cerebral haemorrhage. Here, as there, we have 
to look for direct focal symptoms, and it is to these that most 
attention should be given in our examination ; on the other 
hand, we must not forget that a focus of softening, even if it 
be of considerable extent, may pass through all its phases with- 
out a single symptom. No one region of the brain seems to be 
more exposed to softening than another. We found that the 
number of haemorrhages at the base largely preponderated over 
those in the cortex ; in embolism this is not the case. It is only 
because the surface covered by the cortex is much larger than 
that of the brain stem that we find in the latter numerically 
fewer cases of softening than in the cortex (Wernicke). The 
thalamus and pons are only rarely the seat of isolated soften- 
ing, while haemorrhages are found there much more frequently, 



P SE UD 0-B UL BA R PARAL YSIS. 



249 



whereas the medulla oblongata is more commonly the seat of 
softening (cf. Berlin, klin. Wochenschr., 1891, 24). To diag- 
nosticate haemorrhage in the medulla oblongata during life is 
practically impossible, as in these cases death is almost instan- 
taneous. 

Prognosis. — The prognosis in embolism is, ceteris paribus, 
in general better than that of haemorrhage. Not only is the 
outlook for complete recovery more favorable even if the 
attack has been severe and has lasted for a considerable time, 
but in most cases the danger to life is far less than in apoplexy. 

Indirect action upon the medulla oblongata, in consequence 
of which the urine may contain albumin or sugar, is a rare 
occurrence. Even a softening of considerable extent may ex- 
ist for a relatively long time without the manifestation of any 
grave general symptoms. Yet an unfavorable turn is not im- 
possible, and this should always be feared if a sudden and 
marked elevation of temperature takes place. 

Treatment. — The treatment is very limited; indeed, embo- 
lism as such, and the necrosis produced by it, are entirely out of 
its reach. It can only be directed against the attack or consist 
of the prophylactic measures by which we may hope to prevent 
the occurrence or repetition of the accident. The latter un- 
doubtedly is the more important, and much can be accomplished 
by repeated local bleeding from the head (Laborde), a proce- 
dure which is also indicated in the treatment of the attack itself, 
as the cerebral circulation is possibly favorably influenced by 
it. That absolute rest is strongly indicated in cases where heart 
disease exists, needs hardly to be mentioned. Where there is 
a reasonable suspicion of syphilis, potassium iodide, 2.0 to 5.0 
(grs. xxx-lxxv)/r# die, ought to be exhibited. 

Where there are multiple foci of softening the symptoms 
naturally depend on their seat. At the autopsy a number of 
such foci may be found which could not be diagnosticated 
during life because they were too small and were situated in 
so-called indifferent places. If several portions of the brain are 
affected, each of which gives rise to a focal symptom, there may 
be a complication of the most varied clinical manifestations. 

Of great practical interest is the observation to which of 
late years attention has repeatedly been called, namely, that 
foci of softening may occur in that cerebral portion of the 
cortico-muscular tract which contains the fibres destined to 



250 



DISEASES OF THE BRAIN PROPER. 



supply the muscles used in speaking and swallowing. These 
fibres pass from the lower third of the central convolutions, 
where the supposed centres for the hypoglossus and facial are 
situated, and end in the nuclear region of the medulla ob- 
longata. Such foci have again and again been found, Some- 
times they were bilateral and situated in the basal ganglia, 
especially the lenticular nucleus, sometimes on one side only — 
■e. g., in the right corpus striatum — and it has been observed 
that they sometimes give rise to a complication of symptoms 
which simulate most closely those of Duchenne's bulbar paral- 
ysis. The fact, however, should be especially emphasized that 
the occurrence of such a focus on one side is sufficient by itself 
to produce all these symptoms (Lepine and Kirchhoff, cf. lit.). 

The disturbances which go to make up the clinical picture 
are at times exclusively, always chiefly, referable to speech 
and deglutition. They resemble at first sight so much those 
of bulbar paralysis that the name pseudo-bulbar paralysis, or 
paralysis glosso-labio-pharyngea cerebralis, seems justifiable. 
Still, there are some points which should help us to avoid mis- 
takes. Thus, while the beginning of the true bulbar paralysis 
is slow and gradual, the cerebral form often sets in quite sud- 
denly with apoplectiform symptoms ; in the pseudo-bulbar 
paralysis there is a manifestation of other cerebral disturbances 
which do not occur in Duchenne's disease. Again, the latter 
runs an uninterrupted progressive course, while in the cerebral 
paralysis long remissions are frequently met with. A certain 
asymmetry of the paralysis, which is especially noticeable in 
the orbicularis oris (Berger), favors the diagnosis of the cerebral 
as opposed to the bulbar affection. Far more important than 
all these points is the condition of the paralyzed muscles, which 
show no atrophy (Lereche, cf. lit.), and of the tongue, which 
also does not become atrophied in the pseudo-bulbar paralysis, 
and hence does not assume the appearance so eminently char- 
acteristic of the true bulbar form. Consequently there are no 
changes to be made out in the electrical excitability, whereas 
in Duchenne's disease reaction of degeneration is the rule. If, 
finally, we add that in the cerebral form the laryngeal muscles 
seem to be not at all or only slightly affected, we have sufficient 
data to solve the question of differential diagnosis in most cases 
satisfactorily (cf. the excellent article by Oppenheim and Sie- 
merling). 

The prognosis with regard to life is just as unfavorable in 






PSE UDO-B ULBA R PAPAL YSIS. 



251 



the one as in the other form, only this should be borne in mind, 
that in the pseudo-bulbar paralysis remissions may occur ; that 
we therefore can with a clear conscience give the patient good 
hopes of improvement. The duration of the disease may be 
much longer than is ever the case in the genuine bulbar paral- 
ysis. 

The treatment is not so hopeless as in Duchenne's disease. 
The galvanic current intelligently applied, and careful galvan- 
ization of the brain and peripheral faradization of the paretic 
muscles, frequent excitation of the muscles of deglutition, as 
was described on page 149, all may be tried with the justifiable 
expectation of effecting at least a transient, sometimes indeed 
a quite gratifying, improvement. 

LITERATURE. 

Ziegler. Lehrbuch der allgem. und speciellen pathol. Anatomic Bd. ii, Jena, 

1885. 
Polchen. Zur Aetiologie der Hirnerweichung nach Kohlendunstvergiftung, 

nebst einigen Bemerkungen zur Hirnquetschung. Virchow's Arch., Bd. 

cxii, Heft 1, 1888. 
Wachsen. Zur Pathologie der Encephalomalacie. Inaug.-Diss., Breslau, 1887. 
Moebs. Beitrag zur Pathologie der Gehirnerweichung. Inaug.-Diss., Breslau, 

1887. 
Buchholz. Ueber Veranderungen an den Gefassen der Hirnbasis, XIV. Wander- 

versammlung der suddeutschen Neurologen. Arch. f. Psych., 1889, xxi, 2, 

p. 648. 
Mahaim. Ein Fall von secundarer Erkrankung des Sehhiigels und der Regio 

subthalamica. Arch. f. Psych., 1893, xxv, 2. 
Marchand. Zur Kenntniss der Embolie und Thrombose der Gehirnarterien, 

etc. Berl. klin. Wochenschr., 1894, 1. 

Pseudo-bulbar Paralysis. 

Hahn. Ueber Pseudobulbarparalyse. Inaug.-Dissert., Breslau, 1880. 

Wernicke, loc. czl., pp. 208 et seq., 1881. 

Kirchhoff. Arch. f. Psych, und Nervenkrankh., p. 132, 1881. 

Ross. Brain, July, 1882. 

Berger, O. Paralysis glosso-labio-pharyngea cerebralis (Pseudo-bulbar paraly- 
sis). Bresl. arztl. Zeitschr., 3 et seq., 1884. 

•Oppenheim und Siemerling. Die acute Bulbarparalyse und die Pseudo-bulbar- 
paralyse. Charite-Annalen, 1887, xii, p. 331. 

Schwalbe. Deutsche med. Wochenschr., 1888, 35. 

Lereche. Etude sur la paralysie glosso-labiee cerebrale a forme pseudo-bulbaire. 
Paris, 1890. 

Miinzer. Zur Lehre von der Pseudo-bulbarparalyse. Prager med. Wochen- 
schrift, 1890, 29, 30. 

Galavielle. Des paralysies pseudo-bulbaires d'origine cerebrale. Montpellier, 
i8 9 3- 



252 DISEASES OF THE BRAIN PROPER. 

3. Endarter litis {Syphilitica). 

This process, first accurately described by Heubnerin 1874, 
affects more especially the vessels at the base of the brain. 
The walls become opaque, show grayish translucent or whitish 
thickenings, and the vessels may finally be converted into firm, 
grayish-white cords. The new tissue which encroaches upon 
the lumen of the vessel either originates in the intima by an in- 
crease of the endothelial cells, which become transformed into 
connective tissue (Heubner), or is derived from the nutrient ves- 
sels of the media and adventitia, and consists therefore of emi- 
grated cells (Baumgarten). On account of this tendency to 
thickening and consequent obliteration of the vessels, C. Fried- 
lander has proposed for the process the name endarteriitis ob- 
literans. While not denying that Heubner, who has studied 
the question most carefully, has arrived at important results, 
we must at the same time affirm that the arterial disease, which 
he describes as specific in nature, is not peculiar to syphilis, 
but that we find the same changes wherever we have a chronic 
inflammatory process with the formation of granulation tissue, 
as, for instance, as a consequence of alcoholism (C. Fried- 
lander). This one fact remains of the greatest practical impor- 
tance, that in the course of syphilis the cerebral arteries are 
very frequently diseased, and that as the outcome of this dis- 
eased state the most diverse cerebral symptoms may arise. 
Chorioretinitis, for example, has been observed by Oswald 
(Deutsche Med.-Ztg., 1888, 86). That under certain circum- 
stances a hemianopia can be the result of such disease is 
proved by the interesting case reported by Treitel and Baum- 
garten (Virch. Arch., Bd. cxi, Heft 2, 1888), where, as a conse- 
quence of gummatous arteriitis obliterans of the arteria cor- 
poris callosi dextra, although the optic nerves were intact, a 
unilateral temporal hemianopia had developed. Furthermore, 
it is to be remembered that often enough an autochthonous 
thrombosis due to this arterial disease gives rise to an attack 
w T hich can not be distinguished from the above-described true 
apoplectic stroke with consequent hemiplegia. If recovery 
takes place in these cases the same thing may be repeated sev- 
eral times, and it is especially in syphilitic diseases of the ar- 
teries that this is relatively frequent. The patient suffers from 
intense paroxysmal headaches, occasionally loses his conscious- 
ness, and presents a transient hemiplegia, but again recovers 



DILATATION OF THE ARTERIES OF THE BRAIN. 



253 



fairly well, until finally he succumbs to a graver stroke. This, 
then, is the usual course which the disease takes. It can, of 
course, only be diagnosticated where the history of syphilis is 
clear. 

The recognition may sometimes be difficult if other cerebral 
symptoms are present, such as speech disturbances, intention 
tremor, decrease in memory, and the like, when we are liable 
to think of multiple sclerosis, or progressive paralysis of the in- 
sane, and it may only be the amenability of the disease to spe- 
cific treatment which will clear up all doubts. This consists in 
the use of bold doses of potassium iodide, 4.0-6.0 (3j-3jss.) a 
day in hot milk until sixteen ounces are taken, and an ener- 
getic course of inunctions — thirty to fifty inunctions of 2.0-2.5 
(gr. xxx-xl) ung. hydrarg. It should be begun as soon as pos- 
sible, as the patient is in no way injured by this procedure, 
while the benefit may be most conspicuous. 

4. Dilatation of the Arteries of the Brain. 

Aneurisms of the cerebral arteries may be of traumatic 
origin or, what is more common, may depend upon endarteri- 
itis, and in this latter case syphilis again deserves special men- 
tion, as among fifty cases of brain syphilis there were found 
six instances with aneurisms (Heubner). Spillman reports 
fifteen cases in which following syphilis aneurisms of the 
basilar artery were found (Ann. de Dermat. et de Syph., 1886, 
vii, p. 641). Further, there is the embolic origin of aneurisms, 
which must not be forgotten (Ponfick). 

Dilatations have been noted in the basilar artery, in the 
middle cerebral, and, though but rarely, in the vertebrals. 
Three cases of basilar aneurism have been reported by Noth- 
nagel (Topische Diagnostik, p. 526) ; others by Watson (Lancet, 
October 13, 1888, p. 719). The symptoms presented nothing 
characteristic, but varied much, and even symptoms referable 
to the pons were not in all cases present. Vertebral aneurisms, 
as described by Cruveilhier, Lebert, and others, have occa- 
sionally been found to be attended with occipital neuralgia. 
Dilatation of the vertebrals produced by atheromatous degen- 
eration may affect the surrounding parts and, as a consequence 
of structural changes produced in the neighborhood of the 
vagus, lead to attacks of twitching in the velum palati and to 
grave respiratory disturbances (Oppenheim and Siemerling). 

Aneurisms of the ophthalmic or internal carotid in the 



254 



DISEASES OF THE BRAIN PROPER. 



cavernous sinus may give rise to a pulsating- exophthalmus, 
which can by appropriate manipulation be temporarily pressed 
back into the orbit. The pulsation of the eyeball, which may 
be propagated to the forehead and temple, is a source of great 
annoyance to the patient. In connection with multiple aneu- 
risms, such as have been observed by Paulicki, for instance, 
existing simultaneously in the basilar, the anterior communi- 
cating, and the middle cerebral artery, epileptiform convul- 
sions and psychoses have been noted. Definite pathognomon- 
ic signs do not, however, exist, and the diagnosis intra vitam 
is only exceptionally made with certainty. According to Ger- 
hardt, there can at times be heard between the mastoid process 
and the thick cords of the muscles of the neck a murmur refer- 
able to the cerebral arteries ; it is systolic or continuous, and 
is heard on one or both sides if the patient refrains from 
breathing or swallowing. Nevertheless, it is rather excep- 
tional that a (small) aneurism of the cerebral arteries is diag- 
nosticated correctly during life. In larger aneurisms, which 
produce characteristic focal symptoms, this will at times be 
easier, especially when serological data — e. g., traumatism — 
are present. 

5. The Neuroses of the Arteries of the Brain (Ancemia and 
Hypercemia of the Brain). 

The vaso-motor nerves of the cerebral and meningeal arter- 
ies arise partly from the cervical sympathetic (Donders and 
Callenfels), partly from certain cranial nerves (Nothnagel). 
They may be excited or paralyzed idiopathically, or reflexly, 
especially from the stomach, and the resulting conditions, 
although as yet only imperfectly understood, are of great 
practical importance. Both stimulation and paralysis are, of 
course, usually only temporary, while in the intervals and in 
the normal state the vaso-motor nerves as well as their centres 
are in a state of moderate tonus. If the stimulation should 
from any cause be more than is necessary to maintain this 
normal tonus, a spasmodic contraction of the smaller arteries 
takes place, the absolute amount of blood in the brain becomes 
diminished, the patient gets pale, complains of dizziness, and 
loses consciousness — in other words, " faints " (acute nervous 
cerebral anaemia). At the same time the heart's action is weak- 
ened, the pulse is small, the face and body are covered with 
cold perspiration, and if this irritation is frequently repeated a 



NEUROSES OF THE CEREBRAL ARTERIES. 



255 



certain predisposition to slight changes in the blood pressure 
becomes gradually established, a condition of things which is 
favored by the mobility of the cerebro-spinal fluid. The at- 
tacks now occur on the slightest provocation, and in the in- 
tervals between them the patient complains of dull headache, 
vertigo, etc., the face at the same time usually being of a pale, 
wax-like color. Certain general diseases, especially chlorosis 
and pernicious anaemia, greatly predispose to these paroxys- 
mal vascular spasms ; in fact, cerebral anaemia is not infre- 
quently one of the symptoms of general anaemia, as it is ob- 
served, for instance, after frequent and profuse bleeding from 
haemorrhoids. 

Among the aetiological factors, certain occupations play an 
important role. Working in lead especially may give rise to a 
chronic vascular spasm, and thus to a cerebral anaemia, which 
is associated with almost constant headache (encephalopathia 
saturnina). 

Tanquerel des Planches, the best modern authority on 
saturnine affections, has described this condition, and it has 
again and again been made the subject of the most careful in- 
quiries. It would be beyond the scope of our present work to 
speak of these in detail ; those interested in the subject will 
find references at the end of the chapter ; suffice it only to say 
here that this saturnine anaemia, if the obnoxious action of the 
metal is continued and the disease is once established, may pro- 
duce in the workers severe cerebral attacks, epileptiform con- 
vulsions, and the like. 

The treatment of acute cerebral anaemia consists primarily 
in placing the patient in an appropriate position — that is, with 
the head low or at about the same level as the feet, so as to 
aid the blood flow to the brain ; the use of stimulants (wine, 
brandy, coffee), occasionally a subcutaneous injection of ether, 
may be indicated. Those who are familiar with the procedure 
may inflate the Eustachian tubes, as Kessel recommends ; this 
" air-douche " is said to be an excellent method of producing 
rapidly an increased flow of blood to the anaemic brain (Laker, 
Wien. med. Presse, 1891, 25). 

For chronic cerebral anaemia galvanization of the brain or 
of the cerebral sympathetic may be tried. As a matter of 
course, attention must also be paid to a possible primary cause, 
and every pernicious aetiological factor removed (change of 
occupation, etc.). 



256 DISEASES OF THE BRAIN PROPER. 

The opposite condition, a paralysis of the vaso-motor nerves, 
produces a dilatation of the cerebral vessels, and thus an im- 
mediate overfilling of the same. This can be demonstrated bv 
ophthalmoscopic examination. Often, but not always, the ves- 
sels of the face share in the disturbance ; the countenance of 
the patient assumes a purplish-red color, he complains of throb- 
bing in his temporals and carotids, of headache, of buzzing in 
the ears (acute nervous hyperemia) — in general, of about the 
same symptoms as we have described in the vascular spasm, 
the only difference lying in the color of the face. It is ob- 
served in certain individuals regularly after the use of quite 
moderate quantities of alcoholic beverages (wine, beer), or, just 
as the anaemia, after emotions, strong bodily or mental exer- 
tions, too much study, etc. ; the abuse of tobacco may also 
give rise to it. 

On account of the very varied manifestations of the affec- 
tion different forms of cerebral hypersemia have been distin- 
guished (Andral, Eichhorst). Thus, a cephalalgia a psychical, 
a convulsive, and an apoplectic form have been described, 
according as either headache or psychical excitement, with in- 
somnia or epileptiform attacks or periods of unconsciousness 
(which latter are not rarely followed by cerebral hemorrhage), 
are the most prominent symptoms. The transition between 
these " forms " is, however, so gradual, and so seldom are they 
sharply defined, that for practical purposes it does not seem 
worth while to make the distinction. We have repeatedly ob- 
served marked contraction of the pupils, while in anaemia they 
are more frequently dilated and react sluggishly. As we have 
pointed out above, simple cerebral hyperasmia may produce 
hemiplegia, which can easily be confounded with the apoplectic 
form (pseudo-apoplexy). 

The treatment is rather unsatisfactory ; it is true we may 
in acute attacks of cerebral hyperemia give early relief to a 
patient by placing him in an appropriate — that is, nearly sit- 
ting — posture, by applying ice-bags to his head, or, finally, by 
free venesection ; but these attacks are so frequently repeated 
in individuals predisposed to them that the question of such 
treatment is not of so much importance as of the adoption for 
months and years of a careful dietetic regime. Besides keeping 
the bowels well open — a thing which should never be omitted 
— the patient must be advised to take enough exercise, even 
practice gymnastics ; he should be cautioned against indul- 



DISEASES OF THE CEREBRAL VEINS. 



257 



gence in heavy, indigestible foods, and, above all, in alcoholic 
beverages. A yearly visit to places like Marienbad, followed 
by a stay in a pure mountain air, moderate but daily excur- 
sions on foot, the occasional use of Carlsbad water under the 
direction of the physician — all these may be prescribed with 
advantage. Much caution should, however, be used with the 
so-called cold-water treatment, which, like sea-baths, may only 
increase the hypersemia. This applies equally to the massage 
treatment, which, unless carried out in accordance with cer- 
tain indications and fixed rules, and under the supervision of a 
competent medical man, often is productive of more harm 
than good in this disease. 

LITERATURE. 

Tanquerel des Planches. Lead Diseases ; with Notes and Additions on the Use 

of the Lead Pipe and its Substitutes. By Samuel L. Dana, Lowell, 1848, 

and Boston, 1850. 
Revillout. Gaz. des hop., 68-71, 1873. 

Berger, O. Berliner klin. Wochenschr., xi, 14, p. 122, 1874. 
Malassez. Gaz. de Paris, 1, 2. 1874. 
Brochin. Gaz. des hop., 24, 1875. 
Lepine. Gaz. de Paris, 47, 1875. 
Hirt. Krankheiten der Arbeiter, iii, 49, 1875. 
Haueisen. Wurtemb. Corresp. Bl., li, 36, 1881. 
Ullrich. Zur Encephalopathia saturnina. Allg. Zeitschr. f. Psych., xxxix, 2, 3, 

1882. 
Charlier. Contribution a l'etude pathogenique du saturnisme cerebro-spinal. 

These de Paris, No. 201, 1882, pp. 45 et scq. 
Schulz, Paul. Ueber Encephalopathia und Arthralgia saturnina. Inaug.-Diss. 

Vratislav, 1885. 
Corning. New York Medical Record, November 13, 1886. 
Langeveld. Hyperhemie chronique du cerveau et de la moelle epiniere. Progr. 

med., 28, 1887. 
Westphal, Alex. Ueber Encephalopathia saturnina. Arch. f. Psych., xix, 3. 

B. Diseases of the Cerebral Veins and Sinuses.— The blood 
from the brain and meninges is carried back toward the heart by the 
internal jugular vein. This vessel emerges from the jugular fora- 
men and after its junction with the external jugular becomes the 
common jugular, which, after it has in turn received the subclavian, 
is called the innominate vein. The two innominates together form 
the superior vena cava. 

Between the two layers of the dura mater there exist spaces 

which convey venous blood but are without valves. These are 

called sinuses. The veins of the cortex empty themselves into the 

longitudinal sinus (sin. falcif. maj.), which terminates behind in the 

17 



258 DISEASES OF THE BRAIN PROPER. 

torcular Herophili (confluens sinuum). The mode in which the veins 
empty into the sinus — namely, in the direction opposite to that of 
the blood current in the latter — produces a slowing of the circula- 
tion, and thus explains the frequent occurrence of coagula in the 
veins of the cortex and the sinus. The deep cerebral veins are col- 
lected into two trunks, which are known as the veins of Galen. These 
again unite into one, the vena magna Galeni. They convey the 
blood from the ventricles to the sinus rectus (perpendicularis), 
which in its turn empties itself into the torcular Herophili. The 
blood from the inner ear goes into the cavernous sinus which is situ- 
ated at the side of the sella turcica ; that from the mastoid cells 
into the lateral sinus, which at the jugular foramen passes into the 
so-called bulb of the internal jugular vein. The veins themselves 
anastomose but little with each other, while the sinuses do so freely. 
It is important to note the communications between the intracranial 
and the extracranial veins — for instance, of the nasal with the ante- 
rior end of the longitudinal sinus, the ophthalmics with the sinus 
cavernosus and the facial veins, etc. — and the communications made 
by the venae diploeticae, for only then can we understand how patho- 
logical processes can extend from the outside of the skull to the in- 
side, and how occasionally we find an external swelling in affections 
of the sinuses. 

Here it is more especially thrombosis with which we have 
to deal, which may occur in the veins as well as in the sinuses. 
The distinction is not always easy in life nor even after death, 
because after death the venous thrombosis may extend into 
the sinus and be taken for a sinus thrombosis. 

If only one vein is affected the mischief may be but slight. 
Usually, however, it takes in one or two of the larger veins, 
which become obstructed during the course of exhausting, 
acute, especially infectious diseases or after an injury, for in- 
stance, a blow on the head. The preponderating number of the 
patients are children, and at times, especially during the hot 
season, quite young children, in which cases a special aetiologi- 
cal datum can not be found. The symptoms are the follow- 
ing : Hemiplegia, ushered in by convulsions and lasting only a 
few weeks, is followed by a permanent weakness, not infre- 
quently bv occasional spasms in the arm. The development 
of the child is then usually faulty, for apart from the occasional 
atrophy in one arm or in one leg or of the whole side, epilepti- 
form convulsions may persist for years, which not rarely have 
an injurious influence on the mental development of the patient. 
In such cases at the autopsy often thrombosis of the longitu- 



DISEASES OF THE CEREBRAL VEINS. 



259 



dinal sinus and of the veins emptying into it is demonstrable. 
In adults, such a thing as a thrombosis of the cortical veins is 
extremely rare. 

Sinus thrombosis may have one of two causes. Either we 
have a general disease which favors the coagulation of the 
blood — as in children profuse diarrhoea, acute infectious dis- 
eases, in old people, tuberculous and carcinomatous processes 
— or neighboring parts, as, for instance, the skull bones or the 
skin of the scalp are diseased (erysipelas), an extension of the 
process becoming possible on account of the communications 
between the extracranial and intracranial vessels above de- 
scribed. We distinguish the true inflammatory thrombosis, 
which affects the lateral, the petrosal, and the cavernous sinus, 
from the so-called marantic thrombosis, which often occurs in 
the superior longitudinal sinus. In both cases the secondary 
symptoms of engorgement, which are especially marked in 
thrombosis of the longitudinal sinus and which manifest them- 
selves in so-called meningeal haemorrhages, are of the greatest 
importance. Such meningeal haemorrhages are found in chil- 
dren (post mortem) as thick coagula distributed over the cor- 
tical motor centres, where they have in life given rise to a 
curious combination of paralysis and spasm, the power of spon- 
taneous movements, however, being retained (Gowers). Choreic 
movements complete the picture which congenital chorea, 
bilateral athetosis, and double spastic hemiplegia present, cases 
which are difficult to interpret and still more difficult to diag- 
nosticate. In these patients, too, the mental development 
remains imperfect, and their irregular movements and contrac- 
tures (often most marked in the calf muscles) give them the 
appearance of helpless cripples. 

The diagnosis of sinus thrombosis can only be made with 
any certainty if to the general symptoms (headache, somno- 
lence, paralyses in the distributions of the cranial nerves) signs 
are added which point to circulatory disturbances peculiar to 
sinus thrombosis. Thus, for instance, symptoms of engorge- 
ment in the ophthalmic veins, manifesting itself by prominence 
of the eyeball, oedema of the lids, congestion of the retina, etc., 
point to obstruction of the cavernous sinus ; oedematous swell- 
ings behind the ear to affections of the lateral sinus, and finally 
symptoms of passive hyperaemia in the nose — epistaxis, marked 
fullness in the veins of the temporal region, in small children 
fullness of the anterior facial veins situated betwen the large 



260 DISEASES OF THE BRAIN PROPER. 

fontanelle and the temples (Gerhardt) — to implication of the 
longitudinal sinus. Pain and swelling of the corresponding 
side of the neck may be significant of a jugular thrombosis, 
etc. All these conditions are, however, but rarely met with, 
and they are more easily found in the books than demonstrable 
in the patient. The duration of a sinus thrombosis varies be- 
tween several days and three to at most four weeks. The 
prognosis is usually unfavorable and the treatment unsatisfac- 
tory and purely symptomatic. 

LITERATURE. 

Powell. Case of Idiopathic Thrombosis of Cerebral Sinus and Veins of Galen 
in a Young Woman. Lancet, December, 1888, ii, 23, p. 11 24. 

Horsley. A Case of Thrombosis of the Longitudinal Sinus, together with the 
Anterior Frontal Vein, causing Localized Foci of Haemorrhage, which 
produced Remarkably Localized Cortical Epilepsy. Brain, April, 1888. 

Mittenzweig. Subdurale Blutung aus abnorm verlaufenden Gehirnvenen. 
Neurol. Centralbl, 1889, 7. 

Ferrari. Obliteration experimentale des sinus de la dure-mere, etc. Arch. ital. 
de biol., 1889, xi, p. 171. 

Zaufal. Zur Geschichte der operativen Behandlung der Sinusthrombose in Folge 
von Otitis media. Prager med. Wochenschr., 1891, 3. (Ligation of the 
Internal Jugular Vein and Opening of the Thrombosed Sinus.) 

Parker (Liverpool). Berliner klin. Wochenschr., 1892, xxix, 10. 

Zirm. Wiener klin. Wochenschr., 1892, v, 26-28. (Sinus thrombosis and 
Exophthalmus.) 

Ehrendorfer. Wiener med. Presse, 1893, xxxiii, 19, 20. (Sinus thrombosis dur- 
ing the Puerperal State.) 

Backlers. Arch. f. Psych, u. Nervenkh., 1893, xxv, 1. 

INFLAMMATORY PROCESSES IN THE BRAIN SUBSTANCE. 

i. Purulent Enceplialitis — Brain Abscess. 

Pathological Anatomy. — Circumscribed pus formations in 
the substance of the brain, which anatomically differ in no way 
from pus formations in other organs, are called brain abscesses, 
and we speak of them as encapsulated and non-encapsulated, 
according as to whether or not they are definitely separated 
from the surrounding tissues by sclerotic thickening. In the 
former, a membrane of connective tissue incloses the abscess, 
which contains a thick pus ; in the latter, disintegrated nerve 
tissue and crystals of cholesterin are found in conjunction with 
the frequently very foetid pus, and the abscess walls are formed 
by a soft layer of brain tissue infiltrated with pus, and sur- 
rounded by areas of yellowish softening and oedema. In the 



BRAIN ABSCESS. 2 6l 

softened areas compound granular corpuscles are found in 
great numbers. The size of the abscess may vary from that of 
a pea to that of an apple, and it may even take in nearly the 
whole hemisphere. The larger the abscess the more marked 
are the signs of increased intracranial pressure, the more flat- 
tened and indistinct the convolutions on the surface of the 
brain, and the drier and more adherent becomes the pia mater. 
Should the abscess break through into one of the ventricles, 
pus may eventually be found in all of them, and the ependyma 
then appear cedematous. If it reaches the surface of the brain 
it may give rise to a diffuse purulent meningitis (Wernicke). 

Etiology. — ^Etiologically, injury is of the greatest impor- 
tance, though it need not necessarily have affected the skull 
itself, but may produce an abscess just as well if confined to 
the soft parts ; in such a case, the inflammation extends through 
the bone, and the infectious material penetrates into the brain 
from the flesh wound. If we have no open wound, no break of 
continuity in the soft parts, then even extensive destructions of 
the brain substance often do not lead to an abscess formation, 
just as in the fractures of the skull healing occurs without sup- 
puration provided that the external air is excluded from the 
injured parts of the brain. 

Besides traumatism, suppuration occurring in the neigh- 
borhood of the brain may cause a brain abscess ; thus, in 
rare instances, it is a purulent parotitis or suppuration in the 
nasal cavity, or, more frequently, caries of the petrous portion 
of the temporal bone or suppuration in the middle ear, which 
becomes the starting point. For years an otitis media may 
persist and be attended with a purulent discharge from the 
external ear without any brain symptoms, but suddenly this 
running may stop, the pus is retained, and probably gives rise 
to the caries of the bone, on account of which the petrous por- 
tion may become so soft that it can be cut with the knife ; a 
brain abscess then develops either in the temporal lobe or in 
one of the hemispheres of the cerebellum. 

Suppuration in the bronchi, putrid bronchitis, bronchiecta- 
sis (Biermer), furthermore, ulcerative endocarditis and pyaemia, 
may also give rise to brain abscesses, which are then desig- 
nated as "metastatic" abscesses. Idiopathic abscesses — that 
is, those in which no aetiological factor could be discovered 
— have been observed by Striimpell in some cases of epidemic 
cerebro-spinal meningitis. 



262 DISEASES OF THE BRAIN PROPER. 

Symptoms. — The symptoms of a brain abscess are divided 
into general and focal. There may, however — and this is of 
much practical importance — be no sign of brain mischief at all. 
A man may not complain of anything worth mentioning, save, 
perhaps, of an occasional headache, and at the autopsy a brain 
abscess be discovered. Quite a number of these cases are well 
authenticated, and there can be no doubt as to their existence ; 
to be sure, we ought not to forget to add that the place in 
which such an abscess is developed must be in a so-called in- 
different region. 

Among the general symptoms the one most constant and 
the most distressing to the patient is headache ; it can by no 
means always be localized, but more frequently affects the 
whole head, and may last with greater or less severity for 
weeks or even months. Occasionally the torture is such that 
the patient, incapable of doing anything, is forced to remain 
quietly in bed, although no other symptoms may be present. 
Very often, it is true, disturbances of the sensorium may ap- 
pear after the headache has lasted for a long time ; a strange 
apathy takes possession of the patient, his sleep is disturbed, 
and his general condition is aggravated if, as is common, 
febrile movements set in, which may be attended with convul- 
sions, which are mostly unilateral. Attacks of vertigo, some- 
times severe enough to cause great anxiety, and sometimes 
only transient, occur, and not rarely there are spells of vomit- 
ing, sometimes lasting for days, and acting very deleteriously 
on the patient. The ophthalmoscopic examination, as a rule, 
does not reveal any fundus changes ; choked disks are only ex- 
ceptionally found, certainly much more rarely than in brain 
tumors. The focal symptoms of cerebral abscess are almost 
exclusively direct. This is a fact which is easily understood if 
we consider their mode of origin ; they are produced either 
by a direct destruction of the brain substance or by the pre- 
ceding oedema and the attendant " preparatory softening " 
(Wernicke), both of which processes are strictly local. At the 
same time we must not lose sight of the fact that the part 
affected by this " preparatory softening " is still capable of 
regeneration. Indirect focal symptoms have only been ob- 
served in cerebellar abscesses ; in such, paralyses of the abdu- 
cens and other nerves have been noted (Wernicke). 

How different focal symptoms show themselves, and which 
are characteristic of lesions of the different parts of the brain, 



BRAIN ABSCESS. 



263 



has been discussed above (page 162 et seq); suffice it here to 
add that abscesses of the so-called motor region produce hemi- 
plegias, which appear in a very characteristic manner — namely, 
step by step. In abscesses of the occipital lobe hemianopia is 
the direct focal symptom which, if properly used, may settle 
the diagnosis. The direct focal symptom of the temporal lobe 
— the crossed deafness — can only rarely be accurately deter- 
mined, as the suppuration of the middle ear, which we have 
shown often to be astiologically connected with brain abscess, 
is mostly bilateral, and as testing of the hearing in patients, 
whose mental activity is somewhat dulled, is very difficult, 
since they are usually unable to appreciate any decrease in 
hearing on one side. In general, we must confess that too little 
attention has been paid to the testing of the hearing, and that 
the examinations have not been made with sufficient care. 

In no one of the few reported cases of abscess of the pons, 
the medulla oblongata, and the cerebellum have direct focal 
symptoms been observed, or at least noted with any certainty; 
the general symptoms, which are mentioned in connection 
with the abscesses of the cerebellum, must be attributed to 
pressure produced by the growing abscess. 

Course. — The disease may pursue its course in one of three 
different ways : 

(1) It assumes from the onset a tumultuous character, 
whether it originate from a traumatism or disease of the mid- 
dle ear. Violent pains — at first local, later spreading over the 
whole head, and lasting from two to four days — together with 
marked elevation of temperature and paroxysms of convulsions, 
are followed by grave disturbances of consciousness. These 
may last for three, four, even eight days, when the patient, 
without regaining consciousness, dies in a restless delirium, 
presenting the picture of one suffering from severe organic 
disease. 

(2) These paroxysmal symptoms lose, after a few weeks, 
their acute character, and become less and less marked ; the 
patient seems to feel better, and he may, indeed, be free from 
all trouble for several months. Even the headache seems — at 
least at certain times — to have vanished. This state of absolute 
(or relative) latency may be of variable duration, and may by 
the inexperienced diagnostician be mistaken for complete re- 
covery, but it is doubtful whether this latter ever occurs. It 
certainly happens much more frequently that after this period 



264 DISEASES OF THE BRAIN PROPER. 

of latency the initial symptoms again make their appearance, 
this time to continue without intermission until death. The 
duration of the whole disease comprises then three to six 
months or more ; it is extremely rare that the period of latency 
lasts for years. 

(3) The onset of the disease is insidious and chronic. The 
patient, who presents slight fever and general symptoms, grad- 
ually becomes emaciated. He complains of headache and dis- 
turbed sleep, and from time to time, apparently without reason, 
is taken with chills ; he begins to have a cachectic appearance, 
and bears on his face the imprint of a grave disease. In such, 
withal very rare, cases our patient is suffering from phthisis 
and the brain abscess is of a tubercular nature. The dura- 
tion of this form, as a rule, does not exceed three or four 
months. 

Diagnosis. — In the diagnosis we may have to differentiate 
between brain abscess, purulent meningitis, meningeal haemor- 
rhage, and brain tumor. If the course of the abscess is very 
acute, as has been described above (eight to ten days), then it 
is often impossible to distinguish it from an acute purulent 
meningitis, an error which is the more excusable when all direct 
focal symptoms which often accompany an abscess are wanting. 
Remissions point rather to the existence of a brain abscess. 

From meningeal haemorrhage, which just as abscess may 
be the consequence of traumatism, it is also distinguished by 
its course. Traumatic meningeal haemorrhages usually give 
rise to epileptiform attacks, which are to be referred to the 
effect of the entrance of the blood between the dura and the 
skull on the motor centres. They are immediately followed 
by a coma, which lasts until death. In abscesses the insensi- 
bility usually lasts only a few hours, and only after a marked 
improvement has again taken place do alarming symptoms 
make their appearance. 

A brain tumor can be differentiated from an abscess by the 
fact that in the former febrile symptoms are absent, while on 
the other hand, in the latter, choked disks, which are a fre- 
quent sign in brain tumor, are only exceptionally noted. The 
course — more especially as regards the remissions, which are 
well marked and often of long duration — is characteristic of 
abscess ; a tumor usually is steadily progressive. Finally, we 
are justified in diagnosticating an abscess if after a protracted 
and varying course the disease suddenly terminates with cer- 



BRAIN ABSCESS. 



265 



tain severe symptoms of collapse and death. When this occurs 
it is probable that an abscess existed which has perforated 
either into the ventricles or to the surface. In cases of trauma- 
tism or in cases in which the cerebral symptoms were preceded 
by an otitis media we should always think first of brain abscess. 

The seat of the abscess can only be determined with any 
certainty if characteristic focal symptoms — for instance, hemi- 
anopia or sensory aphasia — are present. In cases of hemiple- 
gia we can, from the order in which the component monoplegias 
occur, draw a conclusion as to the point of origin of the ab- 
scess. Thus, if at first a paralysis of the leg, together with 
marked sensory disturbances, are the prevailing symptoms, 
and only later the arm and facio-lingual region become affected, 
we may conclude that the abscess is proceeding from behind 
forward, while if the symptoms occur in the reverse order, 
then the frontal lobe may have been the starting point and the 
abscess be extending backward. In cases of traumatism the 
abscess is to be located in very close proximity to the injury. 
Where there is a history of otitis media it usually establishes 
itself in the temporal lobe or the cerebellum. The white mat- 
ter is, in the cerebrum as well as in the cerebellum, by far the 
most common seat. In the brain stem it occurs only very 
rarely, while in this situation, as we have seen, haemorrhage 
and softening are more common. 

Prognosis. — The prognosis is absolutely bad with regard to 
recovery and doubtful with regard to life. We can see from 
what has been said that spontaneous cures, most probably never, 
therapeutic cures quite rarely, take place. It is well to be very 
guarded in giving an opinion as to the duration of life, and we 
should never forget that even during a seemingly excellent 
state of health suddenly grave symptoms may develop which 
lead to a rapid termination. 

Treatment. — Of an effectual treatment we can only speak 
in those cases in which an operation is feasible. Since this — 
trephining of the skull, splitting of the dura, opening of the 
abscess with the knife — must always, however, even if con- 
ducted with the strictest antiseptic precautions, be regarded 
as a grave undertaking, we should only resort to it when the 
location of the abscess has been established with some cer- 
tainty. If this has been done, operative measures are at once 
indicated, and should be carried out without delay, provided, 
of course, that the abscess be in a part accessible to the knife, 



266 DISEASES OF THE BRAIN PROPER. 

which, we need not say, is hardly the case in the basal ganglia, 
the pons, the medulla oblongata, and the cerebellum. 

But, unfortunately, an operation is in the greater number 
of cases not feasible on account of the uncertainty, in the top- 
ical diagnosis. Then our treatment can only be symptomatic, 
and we are confined to local bleeding, hypnotics, bromides, 
etc., which effect but little. For that matter the results of a 
so-called successful operation are not always lasting either, and 
repeatedly one, two, or four weeks after the pus has been 
evacuated an unfavorable outcome has taken place — e. g., in the 
case of Wernicke- Hahn (cf. lit.). 

LITERATURE. 

Wernicke und Hahn. Idiopathischer Abscess des Occipitallappens dutch Tre- 
panation entleert. Virchow's Arch., Bd. lxxxvii, 1882. 

Greenfield. Remarks on a Case of Cerebral Abscess with Otitis, successfully 
treated by Operation. Brit. Med. Journ., 12, ii, 1887. 

Frankel, A. Ueber den tuberculdsen Hirnabscess. Deutsche med. Wochen- 
schr., 18, 1887. 

Sommerville. Analysis of the Urine in Two Cases of Cerebral Abscess. Lan- 
, cet, ii, 12, 1887. (Increase of the earthy phosphates.) 

Link. Traumat. Gehirnabscess. Wiener med. Wochenschr., No. 50, 1887. 

Rossa. New York Med. Record, xxxii, July 5, 1887. (Brain abscess following 
a purulent otitis media.) 

Lacher. Miinchener med. Wochenschr., xxxiv, 33, 1887. 

Barr. Glasgow Med. Journ., xxviii, September 3, 1887. (Very protracted 
course — long intermissions.) 

v. Bergmann. Die chirurgische Behandlung von Hirnkrankheiten. (Brain ab- 
scess, etc.) Arch. f. klin. Chirurgie, Bd. 36, 4, 1887. 

v. Bergmann. Deutsche Med.-Ztg., 1888, 100. (Recovery from brain abscess.) 

Mahr. Wiener med. Blatter, 1888, 22. (Recovery from brain abscess.) 

Crawford-Renton. Cerebral Abscess after Orbital Periostitis. Ophthalm. Re- 
view, 1888, v, 206-208. 

2. Nonsuppurative Encephalitis and its Consequences ("Athetosis"). 

A. IN ADULTS. 

There is no question but that inflammatory processes, acute 
as well as chronic, occur in the brain which show no tendency 
to suppuration, although our knowledge of their pathogenesis 
and their symptomatology is very imperfect. These processes 
take place preferably in quite early childhood, or even during 
intra-uterine life ; only exceptionally may they occur in adults, 
as a consequence of the abuse of alcohol. They are then cir- 
cumscribed inflammatory processes, occurring partly in the 



DIFFUSE CEREBRAL SCLEROSIS. 



267 



cortex, partly in the white matter, which admit of regeneration. 
If larger areas are affected, the tissue becomes shrunken and 
of a distinctly firmer consistence, so that it cuts almost like 
leather. Just how these changes are brought about, in what 
way the nerve fibres of the white matter waste and the con- 
nective tissue increases, which of the two processes is .the pri- 
mary and which the secondary, can not as yet be determined 
with any certainty. Peculiar disturbances in nutrition in cer- 
tain areas of arterial distribution may give rise to defects 
which cause a distinct sinking in of the surface of the brain 
(Kundrat), " porencephaly " (Fig. 74). At times we find a true 




Fig. 74.— Porencephaly. 



cicatricial tissue, which characterizes the terminal process of the 
diffuse cerebral sclerosis. The macroscopic appearance of the 
brain is similar to that in the " induration cartilagineuse " of 
Cruveilhier ; microscopically, the same histological elements as 
are seen in all degenerative processes of the gray and white 
matter of the brain, spider cells, and compound granular cor- 
puscles, are noted (Kast). Marie and jendrassik (cf. lit.) see 
in perivascular changes the chief factor which under certain 
circumstances brings about a lobar atrophy. At times we have 
to deal undoubtedly with the consequences of a uniform arrest 
of development which especially takes in one hemisphere, and 
the anatomical cause for which is not understood. The cir- 
cumscribed inflammatory foci may also be found in both hemi- 
spheres, in which case we speak of a double lobar sclerosis. 



268 DISEASES OF THE BRAIN PROPER. 

The clinical course of the disease is practically unknown. 
Probably there does not exist any well-defined constant clinical 
picture, but the symptoms vary according to the anatomical 
seat of the process. They are symptoms of paralysis or of irri- 
tation, and are partly " cortical symptoms " and partly to be 
referred to disease of the cerebral vessels (cf. Friedmann, Arch, 
f. Psych., 1889, xxi, 2, page 461). In the few cases in which a 
diagnosis could be made during life, apoplectiform attacks, 
rhythmical choreic movements, longer or shorter spells of un- 
consciousness, were observed. The difficulty of grouping and 
correctly interpreting the symptoms is chiefly owing to the 
impossibility of an early diagnosis. Hence it will be the chief 
task of future observers to direct their attention to the initial 
stage, for only after we have once become familiar with the 
development and the anatomical changes in this first stage can 
we hope to elaborate an efficient mode of treatment, which, 
we need not say, at present is absolutely wanting. The irra- 
tional trials with potassium iodide we can certainly not regard 
as such. 

B. IN CHILDREN. 

Cerebral Palsy of Childre7i — Polio-encephalitis {Striimpell). 

Pathological Anatomy. — In view of the comparatively fre- 
quent occurrence of cerebral palsies in children, it is rather 
to be wondered at that so extremely little is known about 
their pathogenesis and their initial stage, more especially with 
reference to the anatomical changes that occur. This may 
perhaps be accounted for by the difficulty, and sometimes 
even impossibility, of making an early diagnosis. At a time 
when we are able to recognize the disease we usually have to 
deal with a process which has already passed through all, or 
almost all, of its different stages. It is the same with the le- 
sions which we find : they in no w T ise explain the exact nature 
of the disease, but only give us an idea of the many various 
wavs in which the brain with its meninges may be altered in 
early childhood as a consequence of the disease, which was 
most probably intra-uterine. General cachexias of the parents 
— e. g., syphilis — may be the cause when the affection begins 
during intra-uterine life ; in this case more than one child may 
have the disease. During the act of birth traumatisms may 
produce unilateral or bilateral cortical haemorrhage. After 
birth, infectious diseases (pertussis, scarlatina, tuberculosis, 



CEREBRAL PALSY OF CHLLDREM. 269 

diphtheria, syphilis) play the most important role in the causa- 
tion. The case which is briefly described on page 277 (Fig. 
78) shows that injuries — from a fall, for example — may also 
precede the affection. We do not, however, know of what 
nature this process is, whether it is a sinus thrombosis, as 
Gowers claims, or an inflammation leading to atrophy, as in 
meningo-myelitis chronica. Neither can we tell whether the 
increase in the connective tissue which has been noted by many 
authors is a primary one, and what part the disease of the ves- 
sels, the thickening of their walls (Hayem, and others), plays 
in the process ; but one thing is certain, that the disease is not 
confined to the gray cortex alone (as Strumpell has assumed, 
and for which reason he has proposed the name polio-encepha- 
litis, analogous to polio-myelitis, cf. lit.), but that the white mat- 
ter as well may be implicated. This is shown by the case pub- 
lished by Kast (cf. lit.), and also by the following observation, 
which was made in my wards, and which I propose to relate 
here in brief, as autopsies in cases of this class are rare : 

Magdalena St., twenty-one years old, coming from a healthy fam- 
ily, was taken sick in her second year with violent fever. Accord- 
ing to her mother's account, she had convulsions for four days and 
four nights. When she wanted to get out of bed after this her left 
side was found to be paralyzed. Inside of three months her condi- 
tion was so far improved that she could walk, although with a limp. 
Gradually the left lower leg became smaller and somewhat curved, 
and she complained of pain in the whole limb. The upper extrem- 
ity did not at first take part in the atrophy ; it was, however, almost 
completely powerless. For two years the convulsions did not re- 
appear; but for the last four years the patient had had, on an aver- 
age, about one epileptiform attack every three weeks, in which she 
bites her tongue and passes her urine involuntarily. The following 
is an extract of the note made on October 25, 1885 : 

Head : Right parietal region painful to percussion ; in the region 
of the left glabella and the hairy part of the scalp, on the same side, 
there are several areas of anaesthesia. Pupillary reactions and move- 
ments of the eye muscles normal. Nothing abnormal in the distribu- 
tion of the facial and hypoglossal nerves. On the right side hearing 
is much below normal, on the left there is complete deafness. On 
the anterior third of the left half of the tongue taste is lost. Uvula 
straight and movements of the soft palate normal. 

Trunk : On the left half of the chest touch and the prick of 
a pin are not perceived ; temperature sense seems decidedly sub- 
normal. 



270 



DISEASES OF THE BRAIN PROPER. 



Upper Extremities : The whole left upper extremity, including 
the hand, is shorter and smaller than the right ; motion of the wrist, 
especially extension, is impaired. The hand is flexed on the fore- 
arm, and only with force can the flexion be overcome. The thumb 
is drawn into the hollow of the hand, the rest of the fingers are 
slightly flexed. Motion in the shoulder joint normal ; in the elbow 
joint extension is slightly impaired. There is a general decrease in 
the sensibility. Electrical reactions are found to be normal for both 
currents, on direct as well as on indirect stimulation. The right upper 
extremity does not show abnormity with regard to development, size, 
mobility, or sensibility. 

The left lower extremity is considerably smaller and shorter than 
the right; sensibility is the same as in the corresponding upper ex- 
tremity ; the same holds for the electrical condition. Tendon and 
skin reflexes are retained on both sides. 

The epileptic attacks continued, and occurred about every sixth 
or eighth day ; the intelligence became more and more impaired. A 
tuberculous process in the left lung was superadded to the already 
existing trouble, and, in consequence of general failure of strength, 
the patient died on March 22, 1886. 

Autopsy : Eighteen hours after death. Extract from the post- 
mortem record : After opening the skull the pia is seen to be con- 
siderably thickened at different places, especially over the right 
hemisphere. In volume the right hemisphere is not much smaller 
than the left ; the anterior and posterior central convolutions on the 
right side, especially in their lower half, are markedly atrophic, the 
gyri are shrunken to about a third of their natural size; the mar- 
ginal and the angular gyrus present the same atrophic condition. 
The upper right parietal lobule is less atrophic, nevertheless the gyri 
are here also remarkably narrow. The portions of the first and sec- 
ond frontal convolutions bordering on the central convolution appear 
also atrophic. On section the gray matter is seen to be considerably 
diminished. 

The ventricles appear markedly enlarged. On frontal sections, 
after Pitres' method, no important changes, with the exception of the 
shrinking, either in the centrum ovale, or in the basal ganglia, can 
be observed macroscopically ; on microscopical examination spider- 
cells and fat-granules are found in considerable numbers not only in 
the gray cortex, but also in the white matter. 

This observation determines us in maintaining with Kast 
the old designation, " cerebral palsy of children," a name by 
which no definite pathological change is implied, and in aban- 
doning the term polio-encephalitis, to which the pathological 
changes do certainly not always correspond, especially as the 



CEREBRAL PALSY OF CHILDREN. 



271 



latter name has already been proposed by Wernicke for the 
disease of the gray matter around the third and fourth ven- 
tricles. 

Symptoms. — The symptoms of this disease-group differ 
according as the pathological process is confined to one hemi- 
sphere or attacks both. 

In the former case the symptoms and the course are so 
characteristic that a correct diagnosis can almost always be 
made during life. The disease usually sets in brusquely, the 
symptoms are violent and can not be overlooked. The child 
is seized with a high fever; soon, sometimes only a few hours 
later, twitchings — at first only in one extremity, later in the 
whole side — appear; at times the whole body may be con- 
vulsed ; this may last, with but slight interruptions, for from 
one to three or even four days, and be accompanied by per- 
sistently high temperature. The symptoms now abate, the 
convulsions become less frequent, but after their disappearance 
the child is found to have lost the use of the limbs of one side 
— hemiplegia infantilis spastica (Benedikt). If an early and 
careful examination be made, a moderate facial paralysis is 
noted, the condition of the extremities being very nearly the 
same as has been described on page 226. The condition of 
the facial nerve in this affection has recently been studied by 
W. Koenig (Deutsche med. Wochenschr., 1893, 42). Here, as 
in the common cerebral hemiplegia, the arm is pressed against 
the thorax, the forearm flexed at right angles with the upper 
arm, the hand flexed and adducted, the fingers bent. The leg 
is slightly flexed at the knee joint, the foot extended ; not un- 
commonly the big toe is in marked dorsal flexion. The sensi- 
bility is, as a rule, not much altered. After several weeks the 
little patient regains enough power to perform the coarser 
movements with the leg, while for a considerably longer time 
the arm does not take part in the improvement. If the child 
was able to walk before the onset of the disease, it will gener- 
ally regain this faculty after a time, but its gait will always be 
halting. 

The further course of the disease is not the same in all 
cases, and it has been our experience that it differs according 
as the initial convulsions continue or cease. This, therefore, is 
an important point to consider in the prognosis for the relative 
recovery. It will also decide the question whether the child, 
while bodily more or less a cripple, is in addition to be men- 



272 DISEASES OF THE BRAIN PROPER. 

tally defective and totally useless to the community. What 
conditions determine the continuance of the convulsions, 
whether this is influenced more by the nature of the lesions 
or more by their seat, we are unable to say. 

As a rule, the attacks, even if they should have a tendency 
to continue, do not recur for months, for one, two, or even 
four years, after the acute period of the disease has passed 
off. Then, however, they may return on any provocation — 
after a fright, maltreatment, sometimes during the second den- 
tition — at first at long intervals of months, then more frequentlv. 
At first they may be slight and of short duration, then more 
severe, until finally they resemble in every point the classical 
attacks of epilepsy — in other words, the hemiplegic or hemi- 
paretic patient has now become an epileptic. As has been 
stated, the influence which these attacks have upon the mental 
development of the child is very detrimental. Much more 
often than is the case in idiopathic epilepsy does the patient 
become weak-minded. The condition of speech found in this 
disease is interesting. If the patient had fully acquired speech 
previous to the attack, it is only affected if the lesion is on the 
left side of the brain, in which case the svmptoms do not differ 
from those which we have described under left-sided cerebral 
haemorrhage. If, on the other hand, the patient has not yet 
learned to speak, he will, in case the fits continue, either not 
learn at all or only very imperfectly, and his talk will, even if 
his mind is only slightly impaired, be quite unintelligible; but 
often the attacks do not recur, so that the mental development 
progresses normally. In such cases speech likewise reaches a 
gratifying degree of development even if it had not yet been 
fully acquired or had been lost. The healthy hemisphere takes 
on vicariously the work of the injured one (cf. page 182, remarks 
on aphasia of children). 

Independently of the epileptiform attacks, there may occur 
changes in the extremities which are in a way analogous to 
those described above. An especially characteristic symptom 
is the pronounced spastic condition which manifests itself in 
an increase of the reflexes, rigidity and spasm of the muscles 
— hemiplegia infantilis spastica. This rigidity is especially 
well marked in the muscles of the hand and the calves, and 
leads, preferably in the former location, to contractures, which, 
however, differ from others, inasmuch as they cease during 
rest and sleep and only appear on voluntary motion. Bene- 



CEREBRAL PALSY OF CHLLDREN. 



273 



dikt, above others, has pointed out that at one time one, at 
another time another, group of muscles may be affected ; that, 
e. g., in walking, the foot may be held normally, while again 
in the same foot we may see a talipes calcaneus, or at another 
time a talipes equinus. Similar conditions are observed in the 
hands ; thus the fingers, which appear to be in a state of immo- 
bile flexor contraction, may at other times present a remarkable 
degree of mobility. We shall shortly discuss carefully the 
entirely involuntary movements of the affected hand which are 
noted in the course of this disease. E. Remak has shown that 
such a spastic paralysis with contracture may lead to a luxation ; 
in the case which he reports a retroglenoid subacromion luxa- 
tion developed (Berlin, klin. Wochenschr., 1893, 52). 

In almost all cases of infantile cerebral paralysis an arrest 
in development or growth becomes apparent in the affected 
extremities. This may be only insignificant, so as to be hardly 
appreciable. On the other hand, the limbs may in all their 
dimensions be considerably smaller than the corresponding 
ones of the sound side. Occasionally the whole half of the 
body, trunk and head as well, share in this arrest, and we have 
what is called a general hemiatrophy. Borgherini has ob- 
served this to occur a few days after the onset of the disease 
(Deutsch. Arch. f. klin. Med., xl, 5, 6). 

The following illustrations of cases from my clinic repre- 
sent different types of the cerebral paralyses of children : 

Figs. 75 and 76: Hemiatrophy of the whole left side of the 
bod)'-. Epileptiform attacks. Dementia. 

Figs, yy and 78 : Hemiatrophy of the whole left side of 
the body. Cause : Traumatism. No fits. Intelligence nor- 
mal. 

Fig. 79 : Atrophy of the left upper and lower extremity 
(resection of the knee joint). Epileptiform attacks, with a mod- 
erate degree of dementia. 

Figs. 81 to 84 : Atrophy of the paralyzed side, very slight, 
but perceptible. All three patients suffer from epileptiform 
attacks and are demented. All three present contractures on 
the affected side, either in the wrist (Fig. 81, and also Fig. 75) 
or in the ankle joint (Figs. 83 and 84). 

All these eight cases, which came under my observation, 
depended upon disease of the right hemisphere. Whether this 
side is altogether more frequently attacked, and, if so, how the 
fact is to be explained, 1 dare not at present decide. 
18 



274 



DISEASES OF THE BRAIN PROPER. 



If both hemispheres are affected, the symptoms are very 
different, and the diagnosis is much more difficult. Freud has 
classified all these affections as instances of cerebral diplegia, a 
term which may be accepted without reserve, as it is purely 




Fig. 75- 

descriptive, and is noncommittal so far as regards the patho- 
logical process. He divides the cases into four groups : i. The 
general cerebral spasticity first described by Little and called by 
the English authors Little's disease. 2. The paraplegic spas- 
ticity (so-called spastic paralysis). 3- The bilateral hemiplegia. 
4. The bilateral chorea and athetosis. The latter will be referred 
to again (page 284). Little's disease and spastic paralysis are 
frequently congenital ; in the latter affection especially predis- 
posing and hereditary influences play some part, and several 
members in the same family may develop the disease (Newmark, 



CEREBRAL PALSY OF CHLLDREN. 



27$ 



A Contribution to the Study of the Family Form of Spastic 

Paraplegia, Amer. Journ. Med. Sci., April, 1893). (Cf. Fig-. 80.) 

Diagnosis. — The diagnosis of the unilateral affection is, as a 

rule, easy, as the acute onset with the consequent hemiplegia 





Fig. 76. 
The patient, who is now thirty-six years old, was taken ill in early childhood 
with an acute violent fever and convulsions ; the latter lasted for several days, 
but after that disappeared. From that time the left side did not develop as well 
as the right, so that now the left upper extremity, which can be moved, with 
difficulty only, in the shoulder and elbow joints, is seven centimetres shorter 
than the right, while the left lower extremity is three centimetres shorter than 
its fellow. The whole half of the body has shared in the atrophy, which is also 
well marked in the nates. The circumference of the left upper arm measures 
four centimetres and a half, that of the left leg three centimetres less than that of 
the corresponding extremity. When the patient was fourteen years old the con- 
vulsions reappeared, and he has still one or two epileptiform attacks a week. 
He is quite demented. 



276 



DISEASES OF THE BRAIN PROPER. 



is characteristic enough ; but if the patient come from a phthis- 
ical family and is himself tuberculous, some doubt may arise. 
We may have a case of tuberculosis of the brain to deal with, 
which sometimes resembles in its onset the cerebral palsy of 
children. High fever and convulsions are not absent, and se- 




Fig. 77. 

vere motor disturbances occur also. The fact, however, that in 
cerebral tuberculosis generally the base of the brain with its 
nerves, especially the oculo-motor and abducens, are implicated, 
and, further, that it runs a rapid and fatal course, will enable 
us to make a correct diagnosis. 

Spinal and cerebral infantile paralyses can not be con- 
founded with each other if we keep in mind that, in the latter, 
one whole side of the body is affected ; that the muscles are 
rigid, the reflexes increased ; that convulsions occur not only 



CEREBRAL PALSY OF CHILDREN. 



277 



at the onset, but also in the further course of the disease ; that 
the mind becomes impaired, etc. In the spinal form, either 
one limb alone — arm or leg — is affected or both arms or both 
legs, and the reflexes in the paralyzed extremities are lost — 
signs enough to enable us to differentiate between the two 




Fig. 78. 
The patient, now thirty-four years old, fell, at the age of three, from a high 
foot-stool and injured the right side of his head. He lost a considerable amount 
of blood, and was unconscious for quite a long time. Six months after the in- 
jury the atrophy of the left side of the body became apparent, first in the upper, 
then in the lower extremity. With the exception of this atrophy, which has 
now taken in the whole half of the body, including the thorax (compare the left 
with the right mamma), the patient is perfectly healthy. He has never had epi- 
leptiform attacks, there are no hemiathetoid movements, and no psychical alter- 
ations whatever. 



2?8 DISEASES OF THE BRAIN PROPER. 

affections. A hemiplegia due to cerebral haemorrhage can in 
most cases be excluded, owing to its rarity in childhood. Such, 
moreover, would usually not be associated with any muscular 




Fig. 79. 
The patient, now eighteen years of age, had, when six months old, an " apo- 
plectic stroke," and never learned how to walk properly, since the left half of the 
body was paralyzed up to her second year. The power of motion has improved 
to a certain extent ; the left leg, however, and the left arm have remained be- 
hind in development, so much so that the arm is eight centimetres, the leg 
twenty-five centimetres, shorter than the corresponding limb of the right side. 
The shortening of the leg is partly due to a resection of the knee joint per- 
formed thirteen years ago (the reason for which procedure could not be made 
out). Patient suffers from epileptiform attacks, occurring once a month ; they 
last from a quarter to three quarters of an hour, and consist of more or less vio- 
lent convulsions. During these, consciousness is sometimes completely re- 
tained. There is no trace of dementia. 



CEREBRAL PALSY OF CHLLDREN. 



279 



atrophy. In the diagnosis of the bilateral affection we must 
take into consideration the possibility of a multiple sclerosis, 
Friedreich's disease, brain tumor (especially tubercles), menin. 
gitis, and cerebral syphilis. In many cases it is impossible to 
come to a satisfactory conclusion. 

Prognosis. — The prognosis quoad valetudinem is absolutely, 
quoad vitam relatively, unfavorable. Complete recovery is im- 
possible, and has never been observed. If the patient does not 
succumb during the first days of the disease, he will remain a 
cripple all his life, his mental condition being good only in the 
most favorable cases. Under unfavorable conditions he may 
be epileptic and weak-minded, and to a greater or lesser extent 
deprived of the use of his limbs. The utmost we can expect is 
that the diseased side may atrophy only to a moderate degree, 




Fig. 80. 

The family form of spastic paraplegia: a, fourteen years old; b, sixteen 
years old; c, thirteen years old. In the first the disease began at the age of 
seven and a half, in the second at one and a half, in the third at nine. The 
mother had eleven children, eight of whom are living (among them the three 
patients). '(After Newmark, San Francisco.) 



28o 



DISEASES OF THE BRAIN PROPER. 



that the patient may be sound enough in other respects, bodily 
and mentally, and thus be capable of making his own living 
(Figs. 77 and 78). 

Treatment. — The treatment is, on the whole, entirely un- 
satisfactory. Even by the light of an early diagnosis, we are 




Fig. 81. 

The patient is now forty-four years old. The date of onset of the disease 
can not be definitely determined. She suffered from epileptiform convulsions 
from early childhood up to her tenth year ; these have now entirely disappeared. 
At times, however, a " tic convulsif" (in the distribution of the left facial) is 
noted. The development of the left half of the body has been retarded, the 
upper extremity being two centimetres, the lower three centimetres, shorter than 
the corresponding limb of the right side. There is also a dfference of from 
four to five centimetres in the circumference of the limbs of the two sides. 
The shoulder, elbow, and wrist joints are contracted, the first being in a posi- 
tion of adduction, the second in one of flexion, and the third in extension. 
Marked degree of dementia. 



CEREBRAL PALSY OF CHILDREN. 



28l 




Fig. 82. 

The patient, who is now twenty-two years of age, was taken ill with con- 
vulsions in early childhood. They ceased, but after an interval of ten years re- 
appeared in his fourteenth year, and have continued up to the present time, 
being quite severe and recurring frequently. From childhood he has suf- 
fered from a severe motor speech disturbance, and is only able to utter a few 
unintelligible syllables, and that with great effort ; at such times almost all the 
muscles of the body are affected with associated movements. Atrophy of the 
left side is to be noted. The circumference of the left upper arm measures 
three centimetres, that of the left forearm two centimetres, that of the thigh 
four centimetres, and that of the leg two centimetres less than the correspond- 
ing measurements on the right side. The left arm is one centimetre, the left 
leg one centimetre and a half, shorter than the right arm and right leg respect- 
ively. The left hand and fingers are in flexor contraction. Patient is moder- 
ately demented. 



282 



DISEASES OF THE BRAIN PROPER. 



not in a position either to prevent the continuance of the epi- 
leptiform attacks or to ward off the changes in the affected 
extremities, the symptoms of irritation, the atrophy, etc. The 
symptomatic treatment of the epileptiform attacks by the dif- 
ferent bromides and the galvanization of the atrophic parts is 
all that lies in our power, and, unfortunately, little enough is 
accomplished by these means. 

While we do not attempt to give a detailed account of the 
pathology of cerebral diplegias (Freud, Leipzig und Wien, 




Fig. 83. 



1893), two questions must be discussed, namely, (1) under what 
conditions do contractures, (2) under what conditions do cer- 
tain movements, which are independent of the will of the pa- 
tient, develop ? Unfortunately, we are not able to answer these 



CEREBRAL PALSY OF CHILDREN. 



283 



questions satisfactorily. With regard to the first, the idea de- 
serves to be mentioned that the extent of the cerebral lesion 
and the secondary degeneration depending upon it are of some 
significance. 

The same uncertainty exists in regard to the second ques- 
tion. We are not acquainted with the immediate conditions 
which, in the course of the cerebral palsies of children, give 




The onset of the disease can not definitely be fixed, since the mother of 
the patient does not remember it, and the patient herself, who is now eighteen 
years old, is demented and completely deprived of the power of speech. The 
fact, however, that the illness began in early childhood with convulsions is un- 
questioned ; it is, however, not known how long they lasted nor what followed 
them. When the girl was five years old she was not yet able to walk, because 
the left leg was moved only with difficulty, and the foot gradually assumed an 
equino-varus position, which can still be noted. Patient now walks on the 
outer edge of her foot, and the leg is scarcely moved at the knee joint. The 
left upper extremity can be moved voluntarily in the shoulder and elbow joints ; 
the fingers and the hand present athetoid movements, while in the facial mus- 
cles of the left side a marked "tic convulsif " is noted. Marked dribbling of 
saliva. Patient no longer suffers from epileptic attacks, but has from time to 
time periods of excitement, during which she becomes aggressive. 



284 DISEASES OF THE BRAIN PROPER. 

rise to peculiar (unilateral or bilateral) motions in the affected 
extremities, which present the following characteristics : 

The patients are absolutely unable to keep the fingers and 
the toes of the affected side still ; they are in constant motion 
day and night, during waking and sleeping, without interrup- 
tion. If we observe these movements more closely, we find 
them to be relatively slow, rhythmical, and monotonous. The 
fingers seem to be directed with a definite aim, as if they were 
attempting to seize something, and it is easily remarked that 
the normal limits of the movements are exceeded — the fingers 
are hyper-extended, the toes are elevated almost at right angles 
or fasten themselves to the floor like claws, etc. (cf. Fig. 85). 
All this is only possible in consequence of an unusual stretch- 
ing of the ligaments, which also admits of positions of distinct 
subluxation. The will of the patient has hardly any influence 
over these movements, and only in light cases, and then but 
temporarily, may the patient succeed, by firm pressure of the 
affected hand upon the body, or by fixing the fingers with the 
unaffected hand, in restricting a little the abnormal excursions; 
as soon as the mechanical impediment is removed, they will, 
however, begin again with increased vigor. 

The muscles of the forearm present a firmer consistence, a 
certain degree of hypertrophy. The arm feels hard, and the 
surface temperature is 0.5 to i° C. (0.9 to i.8° F.) higher than 
on the opposite, sound, side ; not but what the muscular strength 
is materially lessened and sometimes so much diminished that 
the examination with Duchenne's dynamometer yields aston- 
ishing results. With the affected arm the patient can hardly 
lift five kilogrammes, notwithstanding the apparently good de- 
velopment of the muscles, while with the well arm five to eight 
times as much work can easily be done. In the muscles of the 
lower extremity a similar condition may be noted ; not infre- 
quently the ankle joint takes part in these movements of the 
toes, and, in exceptional cases, the knee joint as well. Other 
muscles than those of the extremities are not affected. 

The first who studied these movements carefully was Ham- 
mond, of New York, in 1871. He gave them the special name 
athetosis (a-rldy/M) and raised them thus to the dignity of a sepa- 
rate disease, which, in our opinion, they never deserve. Athe- 
tosis — and by this we mean the athetoid spasms — does not con- 
stitute a disease, but merely a symptom. It is the expression 
of cerebral affections, the anatomical basis of which is variable. 



THE A THE TO ID MOVEMENTS. 



285 



Only in the rarest instances, one could almost say never, do 
athetoid movements occur alone without anv other symptoms. 
Almost always they are associated with ler disturbances, 




Fig. 85. 

The patient, now twenty-nine years old, was taken at the age of six months 
with an "apoplectic stroke" followed by convulsions, which at first occurred 
at long intervals, later more frequently, viz., about once every two weeks ; they 
presented all the characteristics of epileptiform seizures. Quite early, peculiar 
involuntary movements appeared in the left extremities, more particularly in 
the left arm, which must be considered as athetoid. At regular interval 
fingers are extended and again drawn into the hollow of the hand, this being re- 
peated about fifty times a minute. In the left foot similar, although, of course, 
less pronounced movements, occurring especially in the ankle joint, are noted 
At about the age of five the convulsions reappeared, although occurring with 
diminished frequency, i. e., from three to five times a year. The patient is ex- 
citable, irascible, and at times even violent. Intelligence is normal. 



286 DISEASES OF THE BRAIN PROPER. 

either psychical (the patients are mentally undeveloped, de- 
mented, sometimes of a changeable, irritable disposition) or 
somatic, such as paralyses or spasms in the distribution of 
different nerves — for instance, the facial — contractures, etc. 
Again, the patient may be subject to epileptiform attacks which 
recur at intervals of various lengths. 

If we thus affirm that every athetosis— be it the much rarer 
bilateral form (see above), be it unilateral, the " hemiathetosis " 
— is only to be regarded as a symptom, we are, on the other 
hand, willing to admit that there are individual cases where 
the athetoid movements are such a prominent and dominating 
feature of the case that we may overlook others, or at least not 
be inclined to attribute any importance to them. So it is in an 
instance reported by Gnauck, who speaks of a primary — that 
is, idiopathic — athetosis, but who has noted a simultaneous 
paresis of the facial and a hemiansesthesia of the affected side. 
We can hardly call this an idiopathic affection, but must rather 
look upon it as a prehemiplegic phenomenon (cf. page 218); 
and, similarly, some explanation can be found for the few re- 
maining cases published as "idiopathic" athetoses, some of 
which were congenital. These movements are always a symp- 
tom of cerebral disease. That they are occasionally met with 
in the course of other diseases — e. g., spinal affections, espe- 
cially tabes — there can be no doubt. The pathological changes 
observer in cases which had presented athetoid movements 
during 7 ife, in addition to those found in cases of cerebral pal- 
sies k\{ children, consisted in small foci of softening in the basal 
ganglia, the thalamus (Lauenstein), the corpus striatum (Schulz), 
and in the temporal lobes (Evvald), although we can in none of 
these instances be certain that the lesions found were actually 
the ^ause of the movements. After cerebral hsemorrhage 
where we have a lesion of the internal capsule, in old hemiple- 
gias therefore, hemiathetoid movements are occasionally seen, 
yet, in comparison with the frequency of cerebral hemiplegias 
in adults, these are very rare, certainly much rarer than in the 
so-called infantile hemiplegias. We see, therefore, that cortical 
lesions and lesions of the cortico-muscular tract as well as of 
the basal ganglia may give rise to athetoid movements, al- 
though we do not understand the nexus causalis, if indeed such 
exist. In our opinion, disease of the cortex undoubtedly plays 
the principal part in the causation of athetosis, and we can all 
the more reckon upon the occurrence of athetoid movements 



THE A THE TO ID MOVEMENTS. 



2S7 



if the cortical disease has appeared in early childhood and has 
been either entirely confined to or has affected more particu- 
larly the motor region, the central convolutions, and the adja- 
cent portions. In lesions of the other parts of the brain, espe- 
cially of the basal ganglia, the thalamus, the lenticular nucleus, 
and the caudate nucleus, athetoid movements are only excep- 
tionally developed, the conditions which favor their occurrence 
being then wholly unknown. That there is a cerebral lesion 
which produces no other symptom, whether psychical or so- 
matic, than these movements is unlikely, and consequently, as 
we said, the name " athetosis," as indicating a separate disease, 
can not be held to be justifiable. 

Keeping well in mind, then, the characteristics of the move- 
ments which have just been described, and especially after 
having had occasion to study their peculiarities, one can hardly 
mistake them for anything else. A good point to remember 
is that they continue during sleep, so that the patients have to 
stop or at least impede them by mechanical appliances. 

We shall give up the idea of chorea or hemichorea which 
we might entertain should the athetoid movements be accom- 
panied by facial spasm, if after observation of the patient we 
have been convinced that the movements persist when he is 
asleep. Furthermore, the duration of the disease and the fact 
that it resists all therapeutic measures, more especially the pro- 
tracted use of arsenic, are facts not reconcilable with the diag- 
nosis of chorea. Other points of difference will be found in 
the chapter on the latter disease. 

We possess no specific which will put a stop to these athe- 
toid movements ; their treatment is that of the primary dis- 
ease, and, as this is usually beyond our reach, the outlook in 
athetosis is necessarily very gloomy. If Hammond claims to 
have effected a cure by stretching the median nerve, we may 
be pardoned for asking how long this cure lasted ; and if 
Gnauck has seen the movements disappear after the use of the 
galvanic current and the internal administration of potassium 
bromide, we are justified in assuming that in his case the affec- 
tion was due to a functional disturbance of the motor area. 
What lasting good results can be accomplished by hyoscine, a 
drug which has been used by Erb, I have not as yet been able 
to establish with the material at my disposal. 



288 DISEASES OF THE BRAIN PROPER. 



LITERATURE. 

Striimpell. Ueber die acute Encephalitis der Kinder. Polioencephalitis acuta, 

cerebrale Kinderlahmung. Vortrag gehalten auf der 57ten deutschen 

Naturforscher-Versammlung zu Magdeburg. 
Jendrassik et Marie. Contribution a l'etude de l'hemiatrophie cerebrale par 

sclerose lobaire. Arch, de Physiol., i, 1885. 
Richardiere. Etude sur les scleroses encephaliques primitives de l'enfance. 

Havre, 1885. 
Marie. Hemiplegie cerebrale infantile et maladies infectieuses. Progr. med., 

xiii, 2me ser., No. 36, 1885. 
Bernhardt, M. Ueber die spastische Cerebralparalyse im Kindesalter. Vir- 

chow's Arch., Bd. cii, 1885. 
Bernhardt. Jahrbuch f. Kinderheilk., N. F., xxiv, p. 384, 1886. 
Kast. Zur Anatomie der cerebralen Kinderlahmung. Arch. f. Psych., xviii, 2, 1887. 
Mathieu. Progr. med., 2, p. 29, 1888. (Cerebral Infantile Paralysis as a Con- 
sequence of Traumatism.) 
Wallenberg. Veranderungen der nervosen Centralorgane in einem Falle von 

cerebraler Kinderlahmung. Arch. f. Psych., xix, 2, 1888. 
Benedikt. Berliner klin. Wochenschr., 1888, 52. 
Hoven. Arch. f. Psych., 1888, xix, 3. 
Audry. Les Porencephalies. Revue de med., 1888, 6, 7. 

Schmaus. Zur Kenntniss der diffusen Hirnsklerose. Virchow's Archiv, 1 888, cxiv. 
Pilliet. Arch, de Neurol., September, 1889, 53. 
Striimpell. Ueber primare acute Encephalitis. Leipzig, 1890. 
Sibut. Arch. de. Neurol., 1890, xix, 57. 
Sachs and Peterson. A Study of Cerebral Palsies of Early Life, based upon an 

Analysis of One Hundred and Forty Cases. Journal of Nerv. and Ment. 

Diseases, May, 1890. 
Freud und Rie. Klinische Studie iiber die halbseitige Cerebrallahmung der 

Kinder. Monograph, Wien, 1891. 
Chaslin. Contribution a l'etude de la Sclerose cerebrale. Arch, de med. ex- 

perim. et d'anat. path., 1891, 2. 
Sachs, B. (New York). Contributions to the Pathology of Infantile Cerebral 

Palsies. New York Med. Journ., May 2, 1891. 
Sachs (New York). Die Hirnlahmungen der Kinder. Samml. klin. Vortr., 

N. F. 1892, 46, 47. 

Michailowski. Etude clinique sur l'athetose double. These de Paris, 1892. 

Freud. Zur Kenntniss der cerebralen Diplegien im Kindesalter. Leipzig u. 

Wien, 1893. 

Athetosis. 

Bidon. Essai sur l'hemichoree symptomatique des maladies de l'encephale. 

Revue de med., 1886. 
Bourneville et Pilliet. Deux cas d'athetose double avec imbecillite. Arch, de 

Neurol., xiv, No. 42, 1887. 
Gerling. Ueber Athetosis. Inaug.-Diss., Kiel, 1887. 
Rubino. Contribuzione clinica alio studio dell' atetosi e del paramioclono molte- 

plice. Riforma medica, 258, 1887. 
(Cf. also the text-books of Striimpell, Seeligmiiller, Eichhorst.) 



BRAIN TUMORS. 



289 



BRAIN TUMORS. 

Pathological Anatomy. — Brain tumors may be either sharp- 
ly circumscribed or diffuse, in the latter case taking- the place, 
as it were, of the brain substance proper. The most common 
— e. g., the gliomata, the carcinomata, and the sarcomata — 
occur in both varieties. The clinical manifestations of brain 
tumors depend upon the rapidity of their growth ; this, again, 
upon their anatomical nature. Among the most important and 
the most frequent forms or*tumors must be mentioned : 

The glioma, a form which is peculiar to the central nervous 
system, but is found much more frequently in the cerebrum 
than in the brain stem or the spinal cord. It is formed by 
an increase in the cells of the neuroglia, the axis cylinders in 




Fig. 86.— Glioma Telaxgiectaticum. (After Ziegler.) Frontal section through the 
brain, a, right centrum semiovale. 6, glioma in the left hemisphere. 

the involved region first becoming swollen, and the nerve 
fibers then destroyed. If the newly formed cells are small and 
comparatively few in number, and if their fibril-like processes 
form a dense network, then the tissue of the growth is firm 
and solid ; if the cells are numerous the tissue is softer. On 
section, the glioma looks gray, grayish-red, or yellowish, some- 
times variegated, and if, as is not uncommonly the case, it 
contain areas of haemorrhagic softening, the tumor may be 
filled with opaque more or less fluid masses. The diameter of 
a glioma may measure from three to eight centimetres. The 
19 



290 



DISEASES OF THE BRAIN PROPER. 



transition into the adjoining- substance of the brain may be 
gradual or abrupt, and the tumor appear macroscopically 
sharply defined. The affected part of the brain is enlarged, 
but keeps its normal configuration while the ventricles are 
often dilated (Fig. 86). 

The tumor nearest related in texture to the glioma is the 
sarcoma; it occurs in soft nodes, which, on section, present a 
marrowy, grayish-white appearance. It is seen much more 
frequently at the base than at the convexity of the brain, and 
not uncommonly is found to originate from the dura, from the 
periosteum of the skull bones, or from the skull itself (osteo- 
sarcoma). According to the character of the cells, we distin- 
guish a round-cell sarcoma, a spindle-cell sarcoma, a fibro- 
sarcoma, etc. In size they may vary from that of a walnut 
to that of a man's fist, and may be solitary or multiple. 

The carcinoma, which appears usually in the brain or in 
the dura as fungus durse matris secondarily to carcinoma of the 
breast, lung, or pleura, is found especially in the ventricles as 
a soft tumor (cf. Fig. 87), displacing the neighboring brain sub- 
stance, and giving rise to hydrops ventriculorum. 

Clinically of great importance are the tubercles and the 
syphilomata (gummata), which, although they show macro- 
scopically as well as histologically much similarity, can with 
certainty be distinguished by the presence or absence of the 
tubercle bacilli. They also may be either sharply defined or 
may infiltrate the tissue ; they appear on section as yellowish, 
cheesy tumors consisting in part of granulation tissue. The 
''solitary tubercles," which may reach the size of a hazelnut, 
are single or multiple ; they occur by preference in the pons, 
in the cerebellum, and in the cortex. Syphilomata more fre- 
quently originate in the dura mater, and thence invade the 
brain substance. 

The psammomata, which, coming also from the dura, are 
characterized by calcareous concretions imbedded in them ; the 
cholesteatomata, which on section have a lustre like that of 
mother-of-pearl ; the lipomata, often found in the corpus callo- 
sum ; the enchondromata, which originate especially from the 
bones of the base — all these are clinically of little importance,, 
as they produce, owing to their relatively small size, either 
only insignificant or no symptoms at all. Hence we may well 
omit them in our description. 

At the autopsy we can often demonstrate the consecutive 



BRAIN TUMORS. 



291 



changes produced by a general compression of the brain. The 
skull bones themselves in young people may appear perforated 
and riddled with holes, there may be gaps in the dura or signs 
of inflammatory irritation, certain areas may be rough and 
thickened, presenting a velvety appearance, the convolutions, 
flattened and pressed against each other, have lost their dis- 
tinctness, the pia looks dry and anaemic. Certain alterations 
of shape seem always to occur if the pressure reaches a con- 




Fig. 87. — Papillary Carcinoma in the Third Ventricle. (After Ziegler.) Frontal 
section through the brain, a, tumor with cysts, b, right thalamus, c, lenticular nucleus. 
d, internal capsule, e, caudate nucleus. _/, left thalamus, g, lenticular nucleus. //, inter- 
nal capsule, t, dilated lateral ventricle. 

siderable degree ; thus a pressure in one hemisphere exerting 
itself from above downward changes more especially the shape 
of the insula and the portions of the temporal and parietal lobes 
which cover it in (Wernicke). This effect must be attributed 
not only to the increase in volume of the tumor, but also to the 
increased amount of the fluid in the ventricles, the hydrops 
ventriculorum (internal hydrocephalus) which almost con- 
stantly accompanies tumors. No doubt this internal hydro- 
cephalus itself is due to pressure on the venous trunks in the 
brain, and it occurs, therefore, earlier, and is more marked if 
the large venous trunks coming from the tela choroidea are 
pressed upon by the tumor (Wernicke). 

On the cranial nerves signs of pressure have also been 



292 



DISEASES OF THE BRAIN PROPER. 



noted. The optic tract, the oculo-motorius, the abducens 
(Tiirck) have been found compressed by tightly stretched 
vessels, and an exudation into the sheath of the optic nerve 
has been observed (Leber). In some cases we find a more or 
less widely spread softening in the parts surrounding the 
tumor, in others this may be entirely absent ; if the softening 
is of a haemorrhagic character, this must be attributed to a 
cutting off of the arterial blood supply produced by the cere- 
bral compression and to venous stasis. Sometimes, in the 
neighboring vessels, there develops an arteriitis obliterans with 
its sequelae (C. Friedlander). Cranial nerves in the immediate 
neighborhood of carcinomata and syphilomata are found to be 
infiltrated with the tumor elements (Wernicke). 

Etiology. — The aetiology of brain tumors is entirely ob- 
scure : we do not know in the least whether certain external 
influences increase the predisposition to tumors in the brain or 
not, just as we are entirely ignorant of the aetiology of tumors 
in general. Although the common idea exists that traumatism 
may be the starting point for a new growth, it is difficult to 
understand the connection; certainly, however, this factor 
plays an infinitely smaller part in tumor than in brain abscess, 
and the occurrence of a brain tumor following an injury is 
probably for the most part accidental. No doubt, in some 
kinds, hereditary predisposition must not be disregarded, as 
in carcinomata and tubercles, but even this loses some of its 
significance, because malignant brain tumors, especially car- 
cinomata, are usually secondary, as we have said. Nothing 
remains, then, but to inquire how far age and sex influence 
their occurrence. With reference to the former, it is supposed 
that some brain tumors, such as tubercles, predominate in the 
young, while carcinomata and sarcomata are chiefly found in 
older people ; others — e. g., myxomata and sometimes gliomata 
— are congenital (Virchow). As to sex, older and more recent 
authors (Lebert, Friedreich, Hasse) agree that males are more 
liable to brain tumors than females, and Wernicke has calcu- 
lated that the proportion is about three to two. 

Symptoms. — The symptoms we are wont to observe in 
brain tumors are due to the mechanical influence which the 
tumor exerts by general or local compression of the skull con- 
tents, and, further, to destructive or irritative actions which 
depend upon certain vital peculiarities of the growth, the irri- 
tation mostly accompanying the infective neoplasms. One or 



BRAIN TUMORS. 2 Q$ 

the other of these just-mentioned factors will influence the 
clinical picture of the disease in a more or less characteristic 
manner, and as one or the other is more prominent the whole 
aspect of the disease will vary. 

With reference to the former, the increased intracranial 
pressure, if it appears acutely, we have first a displacement, 
then an increase of tension, in the cerebro-spinal fluid. In 
chronic processes the latter does not necessarily occur, but as 
the skull cavity gradually becomes encroached upon, some of 
the fluid may be absorbed or the brain become atrophic. As 
the intracranial pressure becomes higher the circulation in the 
brain and its membranes is retarded. What is the cause of 
this retardation, whether the diminution in the tone of the 
vessel walls produces such an increase in the tension of the 
cerebro-spinal fluid that by compression a narrowing in the 
capillaries is produced, or whether fluxionary hyperemias come 
into play, we are not able to decide definitely. At any rate, if 
the blood current in the interior of the skull frequently under- 
goes a slowing, there is a tendency to increased transudation 
and lymph formation, and with it a danger of oedema of the 
brain (cf. von Bergmann, Die Lehre der Kopfverletzungen, 
Stuttgart, 1880, pp. 316-364). 

The symptoms to which this increase of the intracranial 
tension gives rise, and which one has frequently the oppor- 
tunity of studying in the course of brain tumors, may be di- 
vided into general and focal. The former, for the knowledge 
of which we have to thank especially Leyden, Manz, and 
Duret, usually appear in a regular sequence and are always 
the same for the same degree of pressure. 

The most conspicuous and earliest to appear is the head- 
ache. The patient complains of nothing but his head, which 
feels heavy and dull. Every movement causes pain, and this 
becomes at times so violent that the patient feels as if he were 
losing his reason. The pain seems diffuse and can not be local- 
ized. It is in front on the forehead, behind over the occiput, 
to the right, to the left ; it torments him everywhere, and the 
lightest tap with the finger anywhere upon his head is intensely 
disagreeable. Sometimes there comes an hour or two of relief, 
although the patient feels by no means well and is never with- 
out pain even in sleep. The seat of this pain which is due to 
the general increase of the intracranial pressure produced by 
the tumor, is not known. It is, however, not likely to be in 



» 
294 DISEASES OF THE BRAIN PROPER. 

the substance of the brain itself, unless it be perhaps in the 
corpora quadrigemina and the thalami. We should rather 
look for its position in the dura, which derives its nerve supply 
from the trigeminus (cf. page 61). If the fibres of this nerve 
are compressed by the tumor in the posterior fossa, then there 
is not the vague pain taking in the whole head, but another 
well-defined headache referred by the patient to the back of 
the head and neck only, a trigeminal or occipital neuralgia 
which is not a general but a focal symptom. This double sig- 
nificance of the headache may become a very valuable point 
in the topical diagnosis. Entire absence of headache is rare, 
and we fail to find this symptom only when the growth of 
the neoplasm is slow. Its occurrence with unwonted vehe- 
mence has repeatedly been noted in aneurisms situated near 
the dura. Occasionally it disappears when definite focal symp- 
toms become established, and it naturally is more obscured in 
the later stages of the disease, when the patient becomes som- 
nolent. Its existence is then only apparent from the fact that 
the half-unconscious sufferer frequently puts his hand to his 
head and moans. 

A second general symptom is afforded by the epileptiform 
convulsions, which either affect the whole body or are confined 
to one side and during which consc ; ousness may or may not 
be completely lost. They are by no means so frequently asso- 
ciated with brain tumors as headache, still their occurrence is 
common enough to be of diagnostic value (cf. Bremer and Car- 
son, Amer. Journ. Med. Sci., September, 1890). They, too, may 
constitute a focal symptom, as is, for instance, not rarely the 
case in cortical tumors of the frontal or parietal lobes, which 
partly exert local pressure, partly irritate the cortex. We 
must not suppose that these two symptoms, although they are 
both of an irritative nature, always go hand in hand. Either 
one or both may be present, sometimes the one as a general, 
the other as a focal symptom. Convulsions occur in about 
fifty per cent of all cases of brain tumors. Well-marked hys- 
teroid convulsions have been observed by Schonthal in a case 
of tumor in the corona radiata of the frontal lobe (Berlin, klin. 
Wochenschr., 1891, 10). 

The psychical changes constitute a third general symptom, 
which, however, disturbs less the patient himself than his 
friends. A certain slowness in thinking is occasionally noticed 
in the patient, at first temporary, but later more constant — an 



BRAIN TUMORS. 



295 



inability to appreciate properly the commonest details of daily 
life which had never been before remarked in him. At the 
same time the features become dull and lose their animated ex- 
pression, his movements slow and awkward, he grows careless 
in all his doings, and this listlessness about everything going 
on around him may be carried to such an extent that he lets 
his urine and faeces pass from him without showing any con- 
cern or attempting to satisfy his needs in a proper manner. 
Gradually he begins to show occasional signs of bewilderment. 
Things that he meets with every day he no longer recognizes. 
His own house seems strange to him, he forgets the way to his 
diningr-room or bed-room, and has to be shown there, etc. He 
even forgets how to read and to write, how to solve the sim- 
plest mathematical problems which would not give the slight- 
est difficulty to an eight-year-old child, and gradually he 
becomes more and more demented, until this condition passes 
into one of deep coma and death. In other cases the intelli- 
gence seems to remain intact for a long w T hile, and only the 
weakness of memory strikes one. The friends of the patient 
become alarmed on noticing that he forgets things which he 
has said or done only one or two days or even a few hours be- 
fore, that he does not remember the visits of the physician 
who comes daily, but complains of not having seen him for a 
long time. Yet although he may be troubled with bodily pain, 
the patient may seem at the same time cheerful, inclined to 
jest, and to look at things from the humorous side, and it is not 
until later that the other mental defects also begin to show 
themselves, and not infrequently the physician is not consulted 
until the friends discover that the patient is no longer capable 
of conducting his own affairs. Actual speech disturbances do 
not usually occur. Certain peculiarities of speech which do 
come on and make it different from that in health are due to 
the extensive loss of memory of the patient, owing to which he 
has difficulty in finding the right expressions, and often mixes 
them up, etc. This makes him uncertain in speaking. He talks 
slowly, and his deliberation becomes quite noticeable. 

In consequence of the increased intracranial pressure, not 
rarely disturbances in the sensorium occur. The patient is in 
a dazed condition, has a constant desire to sleep, and is drowsy. 
The pulse is often slow at first (forty-five to fifty-five beats 
per minute) and irregular, similar to that which we may ob- 
serve in apoplexy. This retardation is finally followed by an 



296 DISEASES OE THE BRAIN PROPER. 

increase in the frequency in the number of beats. In other 
words, the primary irritation has given way to paralysis of the 
vagus. 

Together with the action of the heart, respiration is affected. 
During coma it is deep, slow, and often stertorous ; with the 
continued increase of the cerebral compression it becomes 
irregular and shallow. Deep inspirations are interrupted by 
long pauses, in one of which the patient dies. 

Slight vertigo, sometimes attended with vomiting, is not 
uncommon. The latter, which is cerebral in origin, has certain 
peculiar characteristics. It usually occurs on the slightest 
provocation. It may be provoked by a simple change in the 
position of the body, and often comes on in the early morning 
and without the existence of any stomach trouble. Without 
any retching large amounts of watery clear stomach contents 
are repeatedly thrown up, and after a short while the patient 
feels perfectly well. Sometimes the vomiting is the forerunner 
of apoplectiform attacks, in which the patient may be uncon- 
scious for hours. Such attacks are due to a sudden increase in 
the intracranial pressure, either from haemorrhage into the sub- 
stance of the tumor or from sudden hydrocephalic exudations 
(Wernicke). 

That papillitis is extremely common in brain tumor we 
have said before. We may add here that it may exist without 
headache, for the increase in the intracranial pressure sufficient 
to produce papillitis does not necessarily produce an appre- 
ciable irritation of the dura, and, on the other hand, if head- 
ache exists without papillitis, it is not referable to the cere- 
bral compression but to irritation of the dura. We should 
never forget that papillitis may exist without any visual dis- 
turbance, and hence never omit the ophthalmoscopic examina- 
tion in suspicious cases, no matter whether the patient com- 
plains of trouble with his eyesight or not. Again, the patient 
may only complain of one eye, while the other seems to per- 
form its function normally, and yet profound changes be found 
in either fundus. 

If in the course of a brain tumor the patient develops in 
addition to papillitis an early blindness, then the amaurosis has 
to be interpreted as a focal symptom, and the tumor located in 
the cerebellum, as neoplasms in this situation are usually at- 
tended with very marked internal hydrocephalus, especially of 
the third ventricle, the floor of which becomes distended and 



BRAIN TUMORS. 



297 



presses upon the chiasm situated under it (Tiirck). Moreover, 
early amaurosis may be produced by tumors in the region of 
the corpora quadrigemina, especially those of the pineal gland, 
by basal neoplasms, which, just as those of the pituitary body, 
press on the chiasm and the beginning of the optic tract, or 
which raise the base of the brain from the base of the skull, 
so that the artery of the corpus callosum is made tense and 
compresses the optic nerve (Tiirck). 

The visual disorders which occur in the course of brain 
tumors have been grouped in the following manner by Hirsch- 
berg (Neurol. Centralblatt, 1891, 15): 

(1) Attacks of blindness — epileptiform amaurosis. (2) Per- 
manent visual disorders : 

A. Produced by changes in the brain : a Homonymous 
hemianopia (destruction of one or both visual centres in the 
occipital lobe). ft Crossed hemianopia (tumors in the region 
of the anterior or posterior angle of the chiasm. 7 Bilateral 
hemianopia — total amaurosis. 

B. Produced by changes in the eye-ground : a Enlarge- 
ment of the blind spot (not noticed by the patient), ft Nar- 
rowing of the field of vision. 7 Diminution of the central 
acuteness of vision, due either to anatomical changes in the 
retina or to interruption of the nerve-fibres leading to the 
retina. 

In considering the focal symptoms produced by brain 
tumors we must first of all state that these may be entirely 
absent, just as we have seen is sometimes the case in brain 
abscess. Instances of this kind have repeatedly come under 
observation, and it was on this very account found impossible 
to make a certain diagnosis during life. Absence of both gen- 
eral and focal symptoms is very rare, and only possible when 
the new growth is very limited, and situated at an indifferent 
place. Further, there are symptoms which we are justified in 
taking for focal symptoms, but which are in reality due to the 
general compression. The most important one of this nature 
is hemiplegia. We may in a case of brain tumor find a well- 
marked hemiplegia, which persists without any amelioration, 
and be induced to call it a focal symptom, and yet, to our sur- 
prise, at the autopsy a tumor may be found in an entirely in- 
different area — for instance, in the white matter of the frontal 
lobes — a connection which we could not reckon upon. An in- 



298 DISEASES OF THE BRAIN PROPER. 

stance of this nature I had published in an inaugural disserta- 
tion. This was the case of a man fifty years of age who suffered 
from mitral insufficiency, and w r ho was seized with a grave 
right-sided hemiplegia which persisted unchanged for months, 
associated with speech disturbances. Papillitis could never be 
demonstrated. The case was then supposed to be one of em- 
bolism in the left middle cerebral artery, but at the autopsy a 
round-cell sarcoma the size of a walnut was found in the white 
matter of the frontal lobe, in the pars frontalis media of the left 
hemisphere (Steinberg, Beitrag zur Localisation der Hirntu- 
moren, Inaugural Dissertation, Breslau, 1886). For the hemi- 
plegia to be uncrossed — that is, to be situated on the same side 
as the tumor — is certainly very exceptional ; in our case it was 
crossed. If focal symptoms make their appearance compara- 
tively early, we mostly have to do with basal tumors which 
produce fatty degeneration and gray atrophy of the involved 
cranial nerves, notwithstanding the no inconsiderable power 
of resistance which such nerves possess. Besides the optic 
(unilateral papillitis) and the oculo-motor (ptosis), the fifth, the 
facial, the abducens, and the hypoglossus are then relatively 
frequently affected. Of the fifth, usually only the sensory por- 
tion is implicated ; sensory disturbances in the face, tic dou- 
loureux, later anaesthesia in its area of distribution, occur 
much more frequently than paralysis of the muscles of masti- 
cation. The facial is, on the contrary, affected in its whole dis- 
tribution — a fact which, in conjunction with the reaction of 
degeneration in the paralyzed muscles which also exists, is 
characteristic of the peripheral origin of the paralysis (cf. page 
89). The whole hypoglossus is involved, which causes not 
only the tongue to be protruded to one side, but also leads 
to atrophy in the affected muscles ; swallowing, mastication, 
and speech are necessarily affected. The hypoglossus paral- 
ysis, however, is much rarer than that of the facial. Com- 
bined affections are found : 

(a) Of the olfactory, the optic, the oculo-motor, and the 
first branch of the fifth in tumors of the anterior fossa. 

(b) Of the chiasm, the oculo-motor, the first branch of the 
fifth, and the abducens in tumors of the pituitary body. 

(c) Of the oculo-motor, the patheticus, the chiasm, in tumors 
of the middle fossa, if situated above the dura, of the three 
ocular nerves and the fifth, if situated below the dura ; and, 
finally, 



BRAIN TUMORS. 



2 99 



id) Of the facial, the trigeminus, the auditory, the glosso- 
pharyngeal, the vagus, the accessorius, and the abducens in 
tumors of the posterior fossa. 

Diagnosis. — It is the object of our diagnosis in a given case 
to determine first the presence, then the position, and finally 
the nature of a tumor. The first question can, as is apparent 
from what has been said, by no means always be answered 
with certainty; especially is this difficult if either only general 
or only focal symptoms are present. Among the former, head- 
ache, we have said, plays the most important role. It may last 
for years without any other signs to lead us to suspect a tumor, 
and it is in such instances that we can easily understand how 
this may be mistaken for simple habitual headache or hemi- 
crania, where the pain may also attain an almost unbearable 
intensity. Yet in hemicrania and its allied affections there 
occur remissions, and there are considerable periods of time 
during which the patient is perfectly free from pain ; whereas 
in the course of a brain tumor this never happens. Here we 
find no intervals of relief, but the patient's sufferings are unin- 
terrupted. Moreover, a headache, no matter how severe it be, 
which is materially improved by the exhibition of salicylates, 
bromide, or caffeine, etc., we can hardly refer to a serious 
organic brain disease. If, however, it persists uninfluenced by 
all the ordinary therapeutic measures, this ought to put us on 
our guard, and make us look further for focal symptoms — 
unilateral papillitis, for instance — which may be present; yet 
we should, on the other hand, not lose sight of the fact that 
there are quite a considerable number of cases of pure migraine 
which do not yield to remedies, and which have to be regarded 
as incurable. 

Convulsions, although less often than headache, may be the 
only striking symptom. If they last for months, appearing at 
moderately long intervals, we may, in the absence of any other 
symptoms pointing to a tumor, think of idiopathic epilepsy. 
Here, also, the therapeutic test may throw light upon the 
subject. Large doses of bromide usually diminish the fre- 
quency as well as the severity of epileptic attacks, at least for 
a time, and the favorable influence of the drug is often, indeed, 
quite striking; while if the seizures are due to an organic cere- 
bral lesion, bromides, even if they be continued for a length- 
ened period, have but little effect. Such fruitless trials should 
direct our attention again to the possible existence of a tumor, 



3oo 



DISEASES OF THE BRAIN PROPER. 



and lead us to search for further symptoms which may help 
the diagnosis. 

If the patient complains of nothing further than attacks of 
vertigo and vomiting, if psychical changes, headache, and con- 
vulsions are absent, then the diagnosis remains uncertain, 
because vertigo can be produced by many different causes, and 
cerebral vomiting is met with in affections so different from one 
another that it is simply impossible to diagnosticate a brain 
tumor from these two symptoms alone. They even do not 
necessarily indicate a brain disease, as we may have to deal 
with Meniere's complication of symptoms, with a stomach- 
neurosis, or a spinal disease — e. g., tabes. The gastric crises 
of the tabetics may resemble very closely the attacks of vomit- 
ing in the course of a brain tumor. 

Among the organic diseases of the brain which may be mis- 
taken for a new growth are brain abscess and meningitis. The 
former — the abscess — is almost always associated with febrile 
movements, and rarely with papillitis ; moreover, there are the 
characteristic remissions, so that the patient's general condition 
may be excellent for years. If we keep these points in mind, 
and if we make it a rule never to diagnosticate a brain abscess 
unless we can obtain in the history some serological datum, 
such as an otitis media, traumatism, etc., the differential diag- 
nosis will usually present little difficulty. In meningitis, fever 
is the most important symptom. Papillitis is more frequent 
here than in abscess, and hence of less value in the differential 
diagnosis between tumor and meningitis, yet the early delirium 
and the jactitations are sufficiently characteristic symptoms to 
be of diagnostic value. 

Other diseases to be considered are chronic cerebral sclero- 
sis associated with arterial disease, and lobar sclerosis. The 
absence of grave general symptoms, the usually much slower 
course, the appearance of multiple sclerotic foci, the absence of 
papillitis, are often points enough on which to base a diagnosis. 

Finally, the possibility of confusing brain tumor with pro- 
gressive paralysis of the insane (dementia paralytica) and with 
chronic alcoholism ought to be spoken of. This can, of course, 
only happen in those cases of brain tumor where apoplectiform 
attacks occur, where headache is either absent or only slight, 
where, however, the mental disturbances are marked, and 
where, owing to the defective memory, the alterations in 
speech become a prominent feature of the case. The course 



BRAIN TUMORS. 



3d 



will clear up all doubtful cases. If we are dealing with a 
dementia paralytica we shall not have to wait long for the 
appearance of the characteristic delusions of grandeur, and the 
patient will become bewildered and have transitory periods of 
excitement, whereas, with the tumor, stupor and somnolence 
are developed. In chronic alcoholism tremor and the occur- 
rence of stomach and liver affections are usual. Above all, a 
conscientious use of the history will guard us from an error in 
the diagnosis. 

The seat of the tumor we can only attempt to determine 
when we have reliable focal symptoms to aid us, but, as we 
have observed, such may be absent, and, as it seems, this is 
more especially the case in soft tumors occurring in the ventri- 
cles and sometimes in the frontal lobes, which give rise to 
symptoms of general compression only ; even tumors of the 
fourth ventricle are by no means necessarily associated with 
well-marked and characteristic symptoms, so that often only a 
probable diagnosis is possible (Josef, Zeitschrift f. klin. Med., 
1889, xvi, 3, 4). It is furthermore perfectly certain that a great 
part of the basal ganglia, the lenticular and the caudate nu- 
cleus, also the anterior portion of the thalamus, the corpus cal- 
losum, the fornix, the choroid plexus, and finally the cerebellum, 
with the exception of the vermiform process, may be the seat 
of neoplasms with a complete absence of all focal symptoms. 
On the other hand, tumors of the motor area, of the occipital 
and temporal lobes, of the pulvinar, of the crus, the pons, the 
medulla oblongata, and of the vermiform process of the cere- 
bellum, often manifest themselves clinically by characteristic 
focal symptoms, which we here need not describe, as they have 
been considered above in detail. Wood (Univers. Med. Maga- 
zine, 1889, April, No. 7) reports a case of tumor in the temporal 
lobe running its course without giving rise to symptoms. Suf- 
fice it only to add that destruction of the pulvinar, no less than 
destruction of the occipital lobe, may give rise to hemianopia, 
that an early oculo-motor paralysis points to the existence of a 
tumor in the crus, while severe general symptoms — tonic con- 
vulsions, without the loss of consciousness, staggering gait — 
indicate a neoplasm in the vermis of the cerebellum. Tumors 
of the medulla oblongata may, if general symptoms are absent, 
simulate bulbar paralysis in their course. Vertigo has often 
been noted in connection with such tumors. Other symptoms 
are changeable and uncertain ; sometimes, indeed, there are no 



302 



DISEASES OF THE BRAIN PROPER. 



symptoms at all. Paralysis of the abducens points to the pos- 
terior fossa as the seat of the neoplasm. The affections of other 
nerves, which are important in this connection, have been men- 
tioned above. 

Where we have amaurosis, the pupillary reaction to light 
ought to be carefully examined. Its presence denotes that the 
optic nerve and tract are intact, and the new growth can only 
be situated in the central optic fibres, while if it is absent or 
much diminished we have to deal with a lesion of the optic 
nerve or tract. Even with the existence of papillitis the pupil- 
lary reaction may be present. Then the occurrence of the 
former with the central lesion must be considered as an acci- 
dental coincidence. If we think it possible that the amaurosis 
is due to double hemianopia, we may examine for the so-called 
hemianopic pupillary reaction (described on page 35) to throw 
light upon the question. 

The existence of focal symptoms, however, does not always 
facilitate the diagnosis as much as we might suppose. This is 
especially true if the general symptoms are very grave and 
pronounced. As we have remarked, a hemiplegia must not 
always be taken for a focal symptom, and we must again insist 
that its presence is of no value for the topical diagnosis. We 
need not mention that various disturbances may be produced 
by indirect action which baffle all attempts at a topical diag- 
nosis (cf. the lecture of Jastrowitz, the reference to which is 
given at the end of the chapter). 

The nature of the tumor can in some cases not be deter- 
mined, while at other times it may be very apparent. The 
course of the disease is of less value in this question than, for 
example, the history of the patient's previous diseases ; and the 
fact that certain tumors show preference for certain portions of 
the brain, sometimes also the age of a patient, are likely to 
afford us valuable hints. 

Where syphilis has existed, we have to think of gummata. 
If the family history be one of tuberculosis or carcinoma, brain 
tubercles or secondary carcinoma ought to be considered. A 
chronic cerebral affection in a child, attended with headache 
and convulsions, is strongly suggestive of solitary or of multi- 
ple cerebral tubercles. Tumors of the cortex are more likely 
to be of a syphilitic or tuberculous nature, while those of the 
base are preferably sarcomata ; those of the white matter, the 
centrum ovale, gliomata. 



BRAIN TUMORS. 



303 



Prognosis. — The prognosis in brain tumor is generally un- 
favorable, and death within one or two years after the appear- 
ance of the first symptoms may be predicted. Spontaneous 
recovery is unheard of, and improvement as a consequence of 
treatment is very rare and has only been observed in cases of 
gummatous or tuberculous neoplasms. Here it occurs beyond 
question, consequently the prognosis is much less gloomy in 
these than in other tumors. In general, the course is, in spite 
of all treatment, steadily progressive. The patient's sufferings 
increase in severity, and the agony is only blunted by the dull- 
ing of the sensorium. Death occasionally sets in suddenly, as 
a rule only after a protracted state of marasmus in consequence 
of exhaustion. 

Treatment. — The treatment is in the vast majority of cases 
of no avail. Only in rare instances can we by a systematic 
administration of potassium iodide (5.0 to 8.0 [grs. lxxv to 3 ij] 
daily in hot milk for one and a half to two months) effect a 
noticeable improvement. Whether this is clue to the direct 
action of the iodide on the tumor, or whether only the second- 
ary changes, the softening, the oedema, the accumulation of 
fluid in the ventricles are influenced thereby, we do not know. 
As a matter of fact, however, the improvement does occasion- 
ally occur, and, be it explicitly stated, not only in cases of gum- 
mata, but also in other, malignant, neoplasms. Besides iodide 
of potassium, arsenic seems at times to have a beneficial action, 
yet sufficient positive observations are wanting on this point. 

The question of operative interference, if such appear indi- 
cated, involves the same principles which we have set forth in 
connection with operation for abscess, and which ought to 
guide us here also. Symptoms pointing unmistakably to an 
exudation into the ventricles justify trephining and tapping of 
the lateral ventricles for the purpose of lowering the intra- 
cranial pressure. The posterior fossa is always a sort of a noli 
me tangere (Wernicke). Headache, vertigo, and vomiting are 
to be treated symptomatically. Instructive cases of brain tu- 
mors, in which an operation was performed, have recently been 
published by Erb (Deutsche Zeitschr. fiir Nervenheilk., ii (1892), 

p. 414). 

r } LITERATURE. 

Bernhardt. Beitrage zur Symptomatologie unci Diagnostik der Hirnge- 

schwulste. Berlin, Hirschwald, 1881. 
Heubner. Drei Falle von Tuberkelgeschwiilsten im Mittel- und Nachhirn. 

Arch. f. Psych, u. Nervenkr., xii, 3, 1881. 



304 



DISEASES OF THE BRAIN PROPER. 



Stiiimpell. Em Fall von Gehirntumor mit centraler einseitig-er Taubheit. Neu- 
rol. Centralbl., No. 16, 1882. 
Andry, J. Les tumeurs des plexus chorioides. Revue de med., vi, 1 1 , p. 897, 1 886. 
Steinberg. Beitrag zur Localisation der Hirntumoren. Inaug.-Diss., Breslau, 

1886. 
Heusser. Virchow's Archiv, Bd. no, p. 9, 1887. (On Tumors of the Pituitary 

Body.) 
Taubner. Ibid., Bd. no, p. 95, 1887. (On Lipoma of the Brain.) 
Daly. Brain, xxxviii, p. 234, 1887. (Tumor of the Pineal Gland.) 
Hutchinson. Ibid., p. 223, 1887. (Neoplasms in both Corp. Striata.) 
Brieger. Berliner klin. Wochenschr., No. 47, 1 887. (Case of Sarcoma of the Pia.) 
Birdsall. Philad. Med. and Surg. Reporter, lvi, April 18, 1887. 
Churton. Brit. Med. Journ., May 28, 1887. 

Leclerc. Trois cas de tumeurs intracraniennes. Revue de med., 12, 1887. 
Sokoleff. Gliom des Centralnervensystems. Deutsches Arch. f. klin. Med., Bd. 

45, Heft 4, 5, p. 443, 1887. 
Jastrowitz. Beitrage zur Localisation im Grosshirn und deren praktische Ver- 

werthung. Berliner klin. Wochenschr., xxiv, 49, 50, 1887. 
Schmidt-Rimpler. Arch. f. Augenheilk., xviii, 2, 1887. (Glioma of the Pons. 

The author speaks of paralysis of the ocular muscles and the origin of 

choked disks.) 
Schweinitz. Philad. Med. and Surg. Rep., lvii, October, 1887. (Tumor of the 

Pituitary Body.) 
Osier. Journ. of Nervous and Mental Diseases, 1887, ii, 12. (Cholesteatoma 

of the Third Ventricle.) 
Siemens. Tumoren in der motorischen Region. Berliner klin. Wochenschrift, 

1888, 15. 
Hoppe. Fall von Tumor der Vierhiigel. Inaug.-Dissert., Halle, 1888. 
Kaufmann, A. Vierteljahrschr. fiir gerichtl. Med., Januar, 1888. (Accident 

followed by Brain Tumor.) 
Rath. Arch. f. Ophthalm., 1888, xxxiv, 4. (Tumors of the Hypophysis.) 
Dudley. Brain, January, 1889. (Symptoms of Tumor appearing Three Days 

after Injury.) 
Hafner. Berliner klin. Wochenschr., 1889, 31. (Symptoms of Brain Tumor 

appearing Five Years after Traumatism.) 
Pean, Ballet et Gelineau. Acad, de med. de Paris, Fevr. 19, 1889. 
Nothnagel. Wiener med. Presse, 1889, xxx, 3. (Diagnosis of Tumor in the 

Corpora Quadrigemina.) 
Oppenheim. Arch. f. Psych, und Nervenkrankh., 1889, xxi, 2, p. 560. (Con- 
tribution to the Pathology of Tumors in the Cerebrum.) 
Christ. Deutsches Arch. f. klin. Med., 1890, xlvi, 5, 6. 
Ewald. Berliner klin. Wochenschr., 1891, 10. (Forced Movements in Cases 

of Tubercles of the Brain.) 
v. Hippel. Virchow's Arch., 1891, cxxvi, 1. (Tumors of the Hypophysis.) 
Kuttner. Zur Casuistik der Hirntumoren. Berliner klin. Wochenschr., 1892, 37. 
Giese. Zur Casuistik der Balkentumoren. Arch. f. Psych, u. Nervenkr., 1892, 

xxiii, 3. 
Ackermann. Deutsche med. Wochenschr., 1893, 22. 
v. Bramann. Arch. f. klin. Chir., 1893, xlv, 2. (Extirpation of Brain Tumors.) 



PARASITES OF THE BRAIN. 



305 



APPENDIX. — PARASITES OF THE BRAIN. 

Among- the parasites found in the brain the cysticerci and 
the echinococci are the most important. 

The former — the cysticerci — are found quite frequently at 
the autopsy when their existence during life was not diagnos- 
ticated or even suspected — a proof that they may be present 
without giving rise to any symptoms, or that they may pro- 
duce a clinical picture such as is often due to other causes. 
The cysts, which are rarely single, but mostly multiple, 
amounting as they may to one hundred or more in number, 
have their seat, some in the meninges, some in the substance 
of the brain, in the gray as well as in the white matter; some- 
times they are free in the ventricles. They may be so numer- 
ous that the whole surface of the brain is studded with them. 
Their size may vary from that of a bean to that of a walnut, 
and but rarely exceeds that of the latter. They contain a 
serous fluid. At a place where the cyst wall is somewhat 
thickened are situated the neck and head, the latter often 
deeply pigmented, and to be recognized on closer examina- 
tion by a crown of hooklets 
and suckers. The parts sur- 
rounding the cyst are either 
perfectly normal or in a state 
of inflammatory softening. 
This latter is found as a rule 
only when the cysticercus 
is dead and has undergone 
changes. If the cyst sends 
out diverticula it assumes the 
form of a bunch of grapes, 
and hence is called cysti- 
cercus racemosus (Virchow, 
Marchand). It is estimated 
that the parasites live from 

three to six years. After their death they are changed into cal- 
careous concretions, surrounded by a connective-tissue mem- 
brane, which in their interior contain cholesterine and fat. 

It is impossible to sketch a clinical picture produced bv 
cysticerci in the brain, because this varies, of course, with the 
seat of the cysts. I had occasion in the past few years to ob- 
serve four cases in my clinic, and of these only one was diag- 




Fig. 88.— Cysticercus Racemosus. 
(After Marchaxd. ) 



306 diseases of the brain proper. 

nosticated during- life, and this one not because it presented 
characteristic symptoms, but owing to the history of the pa- 
tient, from which we learned that he was in the habit of fre- 
quently eating- raw pork. In all four cases the patients suffered 
from epileptiform attacks with convulsions, sometimes with, 
sometimes without, loss of consciousness. Two of them were 
in the intervals between the attacks temporarily completely 
bewildered, and were sometimes for hours not able to find 
their way in the ward where they were staying, did not recog- 
nize their fellow-patients — in short, presented conditions which, 
considering the attacks which they were subject to, were 
looked upon as epileptic equivalents. Motor disturbances 
were not observed in any of the cases ; all of them, however, 
complained at times of headache and vertigo. In one case 
three cysts the size of a pea were found imbedded in the left 
lenticular and caudate nucleus, the internal capsule being 
spared, so that the patient had had perfect use of the right 
extremities. In another case there was found a focus of soften- 
ing the size of a pea, in which the calcified remains of a cysti- 
cercus could be demonstrated, in the left half of the middle 
segment of the pons immediately below the middle line, with- 
out there having been during life any noticeable symptoms of 
destruction. » A third case showed, besides numerous vesicles 
imbedded in the gray cortex, cysticerci swimming free in the 
fluid of the ventricles, the amount of which was considerably 
increased. The high grade of hydrocephalus was probably 
responsible for the mental enfeeblement of the patient, a con- 
dition for which during life the epileptic attacks had been held 
accountable ; these, in their turn, were doubtless connected 
with the parasites in the cortex. Cases presenting a course 
which resembles that of the progressive paralysis of the insane 
I have myself not had occasion to observe. According to 
Wernicke, such instances are not rare (Joe. cit., in, 373). 
Michael has described a case in which the presence of a free 
cysticercus in the fourth ventricle gave rise for a considerable 
period of time to a picture simulating diabetes mellitus 
(Deutsch. Arch, fur klin. Med., 1889, xliv, 5, 6). 

Hence it is evident that a diagnosis of cysticerci and echi- 
nococci in the brain can only be made if we know that the 
patient has had a tapeworm, or if we have been able to demon- 
strate cysticerci in the muscles, the eyes, etc. If in such cases 
epileptiform attacks set in, which alternate with conditions of 



PARASITES OF THE BRAIN. 



307 



paresis, and if we are able to exclude syphilis and tuberculosis, 
we are justified in suspecting the presence of parasites, espe- 
cially of cysticerci. 

The aetiology of cysticerci in the brain is that of cysticerci 
in any other part of the body ; they will develop in persons 
who often give the parasites a chance to invade their body, as 
is, for instance, the case with butchers, and hence they occur 
relatively frequently in such individuals. Therapeutics in this 
case is powerless ; we have no means of destroying the parasite. 

Echinococci are usually found in single solitary vesicles 
on the free surface of the brain or the ventricles. Their yel- 
lowish mucoid contents, surrounded by a cyst-wall and a 
connective-tissue capsule, can break through to the outside, 
and be evacuated through the nose, the ears, etc., and a sort of 
spontaneous recovery take place. 

Echinococci of the brain often do not present any peculiar 
symptoms which could be used for diagnosis. The clinical 
picture by which they manifest themselves is usually that of a 
tumor, but when they have perforated to the outside we may 
be able to demonstrate on the protruding tumor fluctuation 
and pulsation. If they perforate into the orbit they give rise 
to oedema of the lids and exophthalmus. Westphal has ob- 
served a case in which over ninety c)'sts were evacuated to 
the outside. 

That actinomycosis may occur in the human brain is shown 
by the publication of Bollinger (cf. lit.), where a tumor in the 
third ventricle is described which contained numerous charac- 
teristic granules. Often the diagnosis remains obscure, as hap- 
pens sometimes also in actinomycotic affections of the lungs ; 
the process in the brain may remain latent (Orlow, cf. lit.). 

LITERATURE. 

Marchand. Virchow's Arch., Bd. 75. Bresl. arztl. Zeitschr., 1881. 

Zenker. Ueber den Cyst, racem. des Gehirns. Erlangen, 1882. 

Soltmann. Cysticercus cerebri multiplex bei einem ijahr. Kinde. Bresl. arztl. 

Zeitschr., No. 20, 1882. 
Brecke. Ueber Cysticerken im vierten Ventrikel. Inaug.-Diss., Berlin, 1886. 
Goldschmidt. Freier Cysticercus im Gehirn. Deutsches Arch. f. klin. Med., 

xl, 3, 4, 1887. (No convulsions.) 
Bollinger. Ueber primare Actinomycosen im Gehirn des Menschen. Munch. 

med. Wochenschr., p. 789, 1887. 
Engel. Ein Fall von Cysticercus beim Menschen als Beitrag zur Diagnostik 

des Cysticercus cerebri. Prager med. Wochenschr., xiii, 2, 1888. 
Gavoy. Cysticerque du cerveau. Encephale, viii, 1, 1888. 



3<d8 diseases oe the brain proper. 

Manasse. Ein Fall von Cyst. Thalami optici. Neurol. Centralbl., 1888, 22. 
Hammer. Zur Casuistik der sogenannten freien Cysticerken in den Hirnven- 

trikeln. Prager med. Wochenschr., 1889, xiv, 21. 
Bitot et Sabrazes (Bordeaux). Etude sur les cysticerques en grappe de l'en- 

cephale et de la moelle chez l'homme. Gaz. med. de Paris, 1890, lxi, 27-30, 

32-34- 
Wiesmann. Correspondenzbl. f. Schweizer Aerzte, 1890, xx, 11. (Cysticercus 

between the Crura Cerebri ; Paralysis of all Four Extremities.) 
Bostrom (Giessen). Untersuchungen iiber die Actinomykose des Menschen. 

Beitrage zur path. Anat. u. allg. Path, von Ziegler u. Nauwerck, 1890, 

ix, 1. 
Orlow. Zur Frage von der actinomykotischen Erkrankung des Hirns und 

seiner Haute. Deutsche med. Wochenschr., 1890, 16. 

CONGENITAL DISEASES HYDROCEPHALUS MENINGOCELE — POREN- 
CEPHALY ABSENCE OF CERTAIN PARTS OF THE BRAIN. 

Our knowledge of the collections of fluid in the brain, 
which are described under the general term of hydrocephalus, 
is, on the whole, very defective, and this is even more true 
of the causes which bring about the abnormal increase. We 
know that the fluid is either contained between the meninges 
or within the ventricles, and speak accordingly of a hydro- 
cephalus externus and internus. We know further that it may 
collect very rapidly or very slowly. In the former case we 
have a hydrocephalus acutus, and in the latter a hydrocephalus 
chronicus. Finally, we know that the conditions under which 
it develops may sometimes exist during intra-uterine life, or, 
again, may appear much later, and we consequently distinguish 
the congenital from the acquired form. But, after all, the dis- 
tinction which we gain by this is only superficial. About the 
exact manner of development of any of these forms there pre- 
vails a great difference of opinion, and the question under what 
circumstances hydrocephalus may develop as an independent 
idiopathic disease can not be satisfactorily answered. There 
is no doubt but that in by far the greater majority of cases we 
have to do with a congenital disease, and, as a matter of fact, 
this form plays in practice the most important role. 

The congenital hydrocephalus is very rarely external, but 
is much more often internal. It may be well developed at 
birth, so that the circumference of the skull measures sixty or 
seventy centimetres or more. The skull bones then are usually 
so thin that their thickness scarcely amounts to that of a sheet 
of paper. The fontanelles and sutures are separated by wide 
gaps. The distention of the ventricles may be so enormous 



// ] 'DR O CEP HA L I ' S. 



309 



that they form a large cavity which is surrounded by brain 
substance one and a half to two centimetres thick. The lat- 
eral ventricles are usually dilated to a much greater extent 
than the third and fourth ; still, these latter may also be mod- 
erately distended. The whole brain, more particularly the 
basal structures, presents the signs of an increased intracranial 
pressure ; they are flattened out, the corpus callosum may suf- 
fer considerably from pressure (Schroeter, Allgem. Zeitschrift 
f. Psychiatrie, 1888, xliv, 4, 5), the commissures are stretched, 




5*r*i||^ J^:s /i 




Fig. 89.— Hydrocephalus (personal observation i. 

the foramen of Monroe is very large, the walls of the ventricles 
are often covered with granulations, the ependyma inflamed 
and in places slightly thickened. The colorless serous fluid, 
the amount of which may be as much as one and a half litres, 
contains 99 per cent, of water, 0.3 per cent, albumin, traces of 
salts, and so forth, and the sp. gr. is 1.004 to 1.006 (cf. Anton, 
Zur Anatomie des Hydrocephalus u. s. w., Med. Jahrb. 84, 
Jahrg. 1888, N. F. iii, Heft 4, p. 125, from Meynert's clinic). 



3io 



DISEASES OF THE BRAIN PROPER. 



The most conspicuous symptom of hydrocephalus is the pe- 
culiar enlargement of the head. This is, however, not always 
apparent in the first weeks. Sometimes one and a half or two 
months may pass before the increase in size begins to be notice- 
able. The circumference of the head, which at birth meas- 
ures forty centimetres, and a year later forty-four centimetres, 
rapidly becomes greater, and every week a half or one centi- 
metre is added to it, so that after a certain time, often only 
after a few months, the head has reached in circumference a 
size which it does not generally attain to before the age of 
puberty — viz., fifty centimetres. If the distention of the skull 
is equal on all sides it becomes spherical, and forms a striking 
contrast to the smallness of the face, which, of course, does 
not take part in the enlargement. If, however, this is more 
marked in the sagittal diameter, the skull assumes a dolicho- 
cephalic form, and its appearance is no less bizarre. This is 
still more accentuated by the enormously enlarged veins which 
as blue cords run over the skull. The eyes are frequently di- 
rected downward. This may depend upon an insufficient in- 
nervation of the eye muscles. The appearance of a child with 
a well-developed hydrocephalus, the enormous head, which, if 
the child is held erect, rolls from side to side, the small trunk 
which with its shrunken limbs looks as if it was only an append- 
age of the head, the idiotic facial expression, are together 
characteristic enough to warrant the diagnosis without any 
further examination, which would reveal various motor dis- 
turbances, spasms of the muscles, and sometimes increased re- 
flexes. It need hardly be stated that the intelligence develops 
only in a very imperfect manner or practically not at all. Most 
of the children never learn to speak or at least only imper- 
fectly. They are not able to play like others, their conduct is 
silly and senseless, their habits are dirty, and they require 
much painstaking care and nursing. In exceptional cases their 
mental development reaches a somewhat higher stage and they 
are able to comprehend certain things, so that under particu- 
larly favorable circumstances, as in a well-conducted home for 
feeble-minded children, it may be possible to give such children 
an amount of knowledge and skill which is quite remarkable. 
The appearance of epileptiform attacks, which are always to 
be anticipated, often greatly interferes with such attempts. 

The course is either chronic or acute. The issue is always 
unfavorable. The children either die during or soon after 



H YDR CEP HA L US. 



311 



birth, or they attain an age of a few months, or finally they may 
live four or five years, while it is very exceptional for them 
to live longer and to reach the age of puberty. If, however, 
this happens, the head ceases to grow and remains of the same 
size or becomes even a little smaller, and the skull ossifies. If 
death occurs in an earlier stage, this happens either during a 
convulsion or comes on gradually as a consequence of general 
marasmus. There is no question but that in face of this affec- 
tion therapeutics is powerless. We may well omit the usual 
inunctions of the skull with mercurial ointment or the painting 
with tincture of iodine, as well as the internal administration 
of iodide of potassium, without any feeling of self-reproach, for, 
often as these measures have been used, rarely has any good 
result from them been seen. Good general nursing of the 
child, later a well-conducted simple instruction as far as this is 
feasible, finally, symptomatic treatment, more especially of the 
more dominating symptoms, as the epileptiform seizures, which 
are best met with bromides, is more rational than any other 
more or less futile measures, not excluding puncture of the 
head and other surgical interference. That we are ignorant of 
the aetiology we have said above, and would only add here 
that the statement that syphilis and alcoholism in the parents 
are predisposing causes, is without foundation. 

The idiopathic hydrocephalus which appears later in life 
may be connected with atheromatous processes and focal dis- 
eases in the brain. Owing to the rarity of its occurrence, how- 
ever, it has been but little studied, and the possibility that even 
in such cases we have in reality to deal with the secondary, 
deuteropathic, hydrocephalus is by no means excluded. 

The secondary hydrocephalus has at times to be attributed 
to disturbances of the circulation, at times to general disorders 
of nutrition. Among the former may be mentioned active hy- 
peremias of the brain, occurring in consequence of the abuse 
of alcohol, and venous stasis, as it is seen in valvular diseases of 
the heart and emphysema. There are, besides, the circulatory 
disturbances caused by circumscribed meningitides, tumors, 
and abscesses, by which, for example, obstruction of the aque- 
duct of Sylvius may be brought about (Seeligmuller). Among 
the disturbances in nutrition there are certain forms of anae- 
mia, general dropsy, phthisis pulmonalis (Callender). The 
affection may run a very acute course and prove fatal in a few 
days. On the other hand, it maybe eminently chronic, and 



312 



DISEASES OF THE BRAIN PROPER. 



then the symptoms need not by any means be characteristic, 
and it may be the more difficult to make a diagnosis, as the in- 
crease in the size of the head is wont not to take place. Some- 
times the symptoms are those of brain tumor ; again, those of a 
spastic spinal paralysis may predominate. 

The so-called hydrocephalus ex vacuo, a form which devel- 
ops in old people under the influence of a general atrophy of 
the brain, must also be looked upon as a secondary hydroceph- 
alus. It is associated with more or less pronounced dementia. 
About aetiology and treatment nothing need be added to what 
has been said on congenital hydrocephalus. 

Under certain circumstances there are found defects in the 
bony skull cap which allow the contents to protrude. By this 
the dura and galea as well as the skin are raised hemispheric- 
ally, constituting what is called a brain hernia or cephalocele, 
and we speak of an encephalocele if the brain substance and 
the pia are both contained in the dural sac, while if only the 
dropsical soft meninges are to be found in it, it is called a me- 
ningocele. Whether a local decrease of resistance of the mem- 
branous skull and defects of ossification, or perhaps abnormal 
adhesions of the meninges with the amnion, are the cause of 
such anomalies has as yet to be decided. Clinically they pos- 
sess no significance. 

The above-mentioned defects (page 267), which we call por- 
encephaly (Heschl), may also be congenital. Some gyri may 
be entirely or partly absent, so that clefts or funnel-shaped 
openings or pits are formed. The defective areas, unless there 
be a communication with the ventricles, are covered with pial 
tissue, and the empty space is filled up with fluid which col- 
lects in the subarachnoidal tissue ; or, again, the neighboring 
convolutions are pressed together over the gap, and instead of 
a hollow we only find a deep cleft (cf. Ziegler, Pathol. Anat.,. 
ii, 636). 

Very remarkable is the fact that certain parts of the brain 
may be entirely absent. This has been observed for the corpus 
callosum, the fornix, the corpora albicantia, the gray commis- 
sure, and others. With reference to the absence of the corpus 
callosum various hypotheses have been put forward. It has 
been thought to be connected with the development of the 
base of the skull and to depend upon the angle which the 
petrous portions of the two temporal bones form with each 



ABSENCE OF CERTAIN PARTS OF THE BRAIN. 



313 



other (Richter, Virchow's Archiv, 106, 1886). Kaufmann has 
described a case where the corpus callosum was completely 
absent and where its formation had never even begun, so 
that the commencement of the disturbance in development 
had to be referred to a time between the third and fourth 
months. In this case the high grade of internal hydroceph- 
alus which was present had to be looked upon as the cause 
(Arch. f. Psych, und Nervenkrank., 1887, xix, Bd. iii, page 769). 
This, in all probability, is more frequently than is generally 
supposed the immediate cause of congenital malformations due 
to arrest of development which is principally the result of 
traumatism during birth, protracted labor, asphyxia in conse- 
quence of compression, etc. Deficiency in the region of both 
fissures of Rolando are especially of interest because they may 
simulate in their clinical manifestations spastic spinal paralysis, 
although the resemblance is somewhat obscured by the simul- 
taneous presence of cerebral symptoms ; and there is, of course, 
every possible gradation, from the pure picture of a spastic 
spinal paralysis in which only the lower extremities are affected, 
to that in which the arms are implicated and cerebral symp- 
toms are well marked. Schultze (Deutsche medicinische Wo- 
chenschrift, 15, 1889) has observed the spastic rigidity in the 
lower extremities in more than one member of the same family 
(cf. Fig. 80). 

Sometimes certain parts of the brain are only imperfectly 
developed. Such a condition has been found in certain gyri, 
the optic thalami, the corpora quadrigemina, the corpora stri- 
ata, and others. Schroter, among other writers, has described 
such a defect in the corpus callosum, which in his case was 
abnormally short (Allgem. Zeitschr. f. Psych., 1888, xliv, 4, 5). 
The cerebellum may also remain very much behind in devel- 
opment, so that under certain circumstances it scarcely attains 
the size of a walnut. The causes of such local malformations 
are usually as obscure as their clinical manifestations during 
life. 



DISEASES OF THE SPINAL CORD. 



What we have said above about the diseases of the brain 
holds good, with certain limitations, also for those of the spinal 
cord. The anatomy of the cord certainly offers less difficulty 
than that of the brain, and, especially as regards the finer 
structure of the organ, has been more minutely examined into 
and is better understood ; but in the physiology there exist still 
so many points, some obscure, some still under discussion, that 
the pathology remains here also very incomplete. To give a 
description of the diseases of the spinal cord, especially when 
questions of its physiology and pathological anatomy are to be 
discussed, is an extremely difficult undertaking, and were it 
accomplished far better than I have been able to do it, would 
still stand in need of a lenient judgment. We shall adopt the 
same arrangement as in our account of the cerebral diseases, 
and divide the subject into three parts. The first will contain 
the diseases of the membranes of the spinal cord, the second 
those of the spinal or peripheral nerves, the third those of the 
white and gray matter of the cord. 
314 



PART I. 

DISEASES OF THE SPINAL MENINGES. 

The spinal meninges are, on the whole, not frequently dis- 
eased alone ; more often the inflammation spreads from the 
(soft) membranes of the brain to the pia of the cord, or from 
the surrounding- structures to the dura spinalis. The one of 
greatest practical interest among the affections of the meninges 
of the cord is the pachymeningitis cervicalis hypertrophica, 
which we shall shortly describe. 

Of the anatomy but little needs to be added to what has been 
said on page 3. The spinal portion of the dura is thinner than 
the cerebral ; it widens into a large cylindrical sac, which is by no 
means filled up by the spinal cord. This dural sac extends beyond 
the lower end of the spinal cord (conus medullaris), and terminates 
in a cone-shaped point at the level of the second sacral vertebra ; 
all these are points too well known to be dwelt upon here at length. 
The conus medullaris ends in the filum terminale, a filiform process 
which is accompanied by the longitudinal nerve bundles coming 
from the lumbar and sacral portion of the cord, which constitute the 
cauda equina. The so-called ligamentum denticulatum is a fiat band 
which by its inner edge is connected with the pia and externally by 
a toothed edge to the dura mater ; the arachnoid lies in such close 
contact with the dura that the subdural space is only a capillary 
space, whereas the subarachnoid space, situated between the arach- 
noid and the pia, is of considerable width. The denticulate ligament 
divides it, though incompletely, into an anterior and a posterior 
half. In contradistinction to the pia mater of the brain, that of the 
spinal cord presents two different layers of connective tissue, the 
outer one of which, very well developed in man, passes into the sub- 
arachnoideal trabecular, while the inner is made up of a single layer 
of circular bundles of fibrillar (Schwalbe) {vide Fig. 90). 

315 



3i6 



DISEASES OF THE SPINAL MENINGES. 



CHAPTER I. 

INFLAMMATIONS OF THE DURA MATER. 

Pachymeningitis Spinalis. 

While in the cerebral portion of the dura the inner surface 
is the usual seat of the inflammation, we find that the spinal 
dura mater may be diseased on its outer as well as on its inner 
surface ; yet the clinical recognition and separation of these 
two forms is very often impossible. 

The inflammation of the outer surface of the dura, the 
pachymeningitis spinalis externa, or the inflammation of the 



Id. 



p. r. 




ad. d.m. i.p. epd.s. 

Fig. 90. —Cross-section through the Vertebral Column and the Spinal Cord- 
(Diagrammatical), epd. s., epidural space, sd. s., subdural space, sar. s., subarach- 
noid space, i. p. , inner periosteum of vertebra, d.m., dura mater, ad. , arachnoid, p. r. , 
posterior spinal root. /. d., denticulate ligament, a. r., anterior spinal root. (After 
Eichhorst.) 



connective tissue between the dura and the vertebral column, 
peripachymeningitis, is a very rare disease, and probably only 
occurs secondarily. The inflammatory changes, which at times 
are most marked on the posterior surface, consist of a thick- 
ening and cellular infiltration of the dura ; sometimes, also, 
the membrane may be found covered with dense cicatricial 
deposits (Eichhorst). The chief causes are caries or tuber- 
culosis of the vertebras, pleuritis, psoas abscess, syphilis, puer- 
peral pyaemia, suppuration in the peritoneal cavity, and in 
exceptional cases the disease may have its origin in a neuritis 



INFLAMMATIONS OF THE DURA MATER, 



317 



migrans. The clinical picture depends largely upon the impli- 
cation of the nerve roots and of the spinal cord. If the cord 
is compressed by the thickening, the symptoms of a pressure 
paralysis, to which we shall have occasion to refer later, make 
their appearance. If the nerve roots are implicated, there are 
violent paroxysmal pains which run along the vertebral col- 
umn and radiate into the extremities. Rigidity of the neck 
and tenderness on pressure over the spinous processes of the 
vertebrae are rarely absent, but are not sufficient to warrant a 
diagnosis, as they may be found just as well in an inflammation 
of the pia. To make a definite diagnosis will in any case only 
be possible if accompanying signs are taken into consideration, 
more especially those of any primary disease. It is always a 
difficult, sometimes even an impossible, task. 

The inflammation of the internal surface of the dura mater 
usually develops in the cervical portion of the cord ; follow- 
ing Charcot, who first described the anatomical and clinical 




Fig. 91.— Cross-section through the Middle of the Cervical Enlargement in 
Pachymeningitis Cervicalis Hypertrophica. th. d., thickened dura, n.f.c, 
newly formed cavities, n. r., nerve-roots. (After Charcot.) 



features of the disease, it has been called pachymeningitis cer- 
vicalis hypertrophica ; the inflammatory new formation and 
thickening of the connective tissue, which are most marked on 
the posterior inner surface of the dura, exist in circumscribed 
areas (Fig. 91); this compresses the nerve roots, which pass 
through the membrane at these places, and finally even the 
cord, and may give rise to the formation of channel-like cavi- 



318 DISEASES OF THE SPINAL MENINGES. 

ties {n. f. c. in Fig. 91). If the compression continues for a 
considerable time it leads to secondary degeneration of the 
pyramidal tracts in the spinal cord, as well as of the motor 
nerves originating in the parts diseased, and to atrophy of the 
muscles supplied by them. 

Symptoms. — The symptoms of the disease are mostly the 
outcome of the participation of the nerve roots and the spinal 
cord. The disease may well be divided into two stages, each 
having its characteristic symptoms. To the first belong the 
pains, to the second the paralyses (Charcot). The pains vary 
extremely in intensity and extent ; as a rule they are confined 
to the region of the neck, whence, occurring in paroxysms of 
increasing severity, they radiate into the upper extremities and 
are accompanied by parassthesias in the arms, tingling and 
formication in the finger tips. The grip is usually markedly 
diminished, and a test with the dynamometer shows that the 
patient is able to lift only ten to fifteen kilogrammes. Not 
rarely trophic disturbances, in the form of vesicular eruptions, 
roughness and desquamation of the epidermis, are noted. The 
sensation of stiffness in the neck and of difficulty in moving the 
head troubles the patient a great deal, and gives to him a stiff, 
quite characteristic appearance. He carefully avoids turning 
his head in any direction, and tries to make up for this rigidity 
of his neck by turning the whole body, which he does slowly 
and in a somewhat awkward way. The most careful examina- 
tion of the cervical region, percussion of the spinous processes, 
hot sponges applied to the skin over them, and the like, does 
not always reveal an increased sensitiveness. 

Gradually, that is to say, in the course of two or three 
months or more, the patient gets accustomed to his pains, so 
much the more so as they become less severe in the further 
course of the disease. On the other hand, he discovers to his 
great distress that the motor power of his upper extremities is 
becoming more and more impaired. The stage of paralysis, as 
a rule, is immediately preceded by a peculiar heaviness and 
stiffness in the shoulder and elbow joints. The patients notice 
that they are unable to raise their arms as high as before ; if 
they are females, that they can not arrange their hair them- 
selves any more, owing to the impairment in the upward and 
backward motion of the arms, movements which finally become 
totally impossible. 

The elbow joint, too, becomes stiffened, and the motions 



INFLAMMATIONS OF THE DURA MATER. 



319 



of the wrist and finger joints become visibly impaired. The 
disability is not, however, usually equal in both arms and 
hands, as one hand may be almost useless, while the function 
of the other is not much interfered with. Still, in some in- 
stances, the trouble may progress in both arms pari passu. 
Curiously enough, not all the muscles of the forearms become 
affected, but more especially those supplied by the ulnar and 
median nerves, while the extensors, which are supplied by the 
musculo-spiral, remain more or less intact. The affection of 
the muscles manifests itself by an increasing atrophy and weak- 
ness, which allows an overaction of the healthy antagonists — 
the extensors — so that the hand, although by no means in all, 
but only in the well-marked cases, assumes a very character- 
istic position. It is dorsally flexed, and the fingers, which are 
bent in the second and third joints, give to it the appearance 
of a claw (Fig. 92). About the development of this position 




Fig. 92.— Position of the Hand in Pachymeningitis Cervicalis Hypertrophic.^. 

(Charcot.) 



we shall have more to say when speaking of the ulnar paraly- 
sis. The difficulties arising from this diminished motor power 
are considerably aggravated by the parsesthesias in the finger 
tips. The patients are unable to take hold of small objects — 
pins, steel pens, etc. — they are unable to attend to their own 
toilet because they can not feel small buttons, and so forth. 
They become more and more helpless, and, what is of the 
greatest significance for patients belonging to the working 
classes, they become incapacitated for work and unable to earn 
their living. This may indeed be the case at quite an early 
period, when the patient is otherwise in a comparatively fair 



320 DISEASES OF THE SPINAL MENINGES. 

condition, especially in female patients who do fine hand-work 
(sewing, knitting, embroidering). The whole condition be- 
comes worse and worse. Arms and hands become stiffer and 
stiffer, until finally, although not in all instances, a complete 
paraplegia of the upper extremities develops. Whether or not 
to these symptoms a paresis or paralysis of the lower extremi- 
ties or bladder disturbances are added will depend entirely 
upon the extent to which the spinal cord takes part in the pro- 
cess. It can in no case either be predicted or excluded with 
certainty. 

Course.— The course of the disease is always chronic and 
extends over years. After the period of pains has passed the 
patients are, as a rule, free from them forever, and only suffer 
from the helplessness which results from the motor disturb- 
ances. Owing to this they require scrupulous care, have to 
be dressed, undressed, fed, etc., by an attendant. Recovery 
or even an improvement is an extremely rare outcome. To be 
sure, I have seen a cured patient in the clinic of Charcot, but 
from the minuteness with which this case was described, from 
the feeling of justifiable pride which accompanied the demon- 
stration, one could well see how extremely rare a cure must be. 
Remak, too, speaks of the curability of the disease (Deutsch. 
med. Wochenschr., 1887, No. 26). I myself am unable to pre- 
sent such a case. The patients in my wards, after unsuccess- 
ful trials of all proposed modes of treatment, have long given 
up all hopes of any marked improvement. 

Diagnosis. — The disease may, especially in its onset, possibly 
be confounded with either spinal leptomeningitis or, as we 
shall later show, with syringomyelia. It is natural that tumors 
of the vertebral column, if they be situated in the region of 
the cervical enlargement, should produce in the initial stage 
the same symptoms as a* pachymeningitis. The further course, 
however, will soon settle the diagnosis. Besides these there 
are two more diseases which may in the mind of the beginner 
give rise to some difficulties with regard to the differential 
diagnosis — namely, progressive muscular atrophy and amyo- 
trophic lateral sclerosis. It is true that a patient with a pachy- 
meningitis may sometimes present the appearance of a man 
suffering from progressive muscular atrophy ; but the two dis- 
eases should never be confounded, inasmuch as in the latter 
affection the initial stage is not accompanied by pain, and the 
stiff neck has never been known to occur in it. The idea of 



INFLAMMATIONS OF THF DURA MATER. 321 

amyotrophic lateral sclerosis will probably also be discarded, 
as in this disease the lower extremities are implicated, and as 
difficulty in swallowing, a sign which indicates extension of the 
process to the medulla oblongata, will usually not be very late 
in appearing. We may say that the diagnosis of cervical 
pachymeningitis can, if the case is carefully examined and if 
the course of the affection is taken into consideration, almost 
always be correctly and definitely made out. 

Etiology. — We are wholly ignorant of the aetiology of the 
disease. Some maintain that the abuse of alcohol is of some 
importance in this connection, others the living in damp houses. 
Whether syphilis has any such influence, and, if so, what is its 
mode of action, is not as yet established. There is no doubt that 
the affection is more common among the working classes and 
the lower grades of society, but what are the conditions and in- 
fluences which act as direct causes, if such there be, we are not 
able to say. 

Treatment. — The treatment comprises local as well as gen- 
eral therapeutic measures. The former consist in the applica- 
tion of strong counter-irritants — e. g., the painting twice daily 
with tincture of iodine, in the use of irritative ointments or 
moxae. The application of Paquelin's cautery, with which 
punctiform scars on the skin are produced (the so-called points 
de feu of the French), only deserves preference because it is 
less painful than the others. Any lasting result can not be ex- 
pected from it. No more is effected by general or internal 
treatment, and it is impossible to give the indications for any 
particular remedy. Iodide of potassium has been used in order 
that something might be done, without, however, producing 
anything else than disorders of digestion. If the patient in- 
sists on taking medicine, a placebo ought to be given — acids, 
bitters, etc. In no case were we able*to see any beneficial 
effects from warm baths and hydrotherapy in general. The 
only measure which at least modified the symptoms somewhat, 
in that it gave the patients for a time more freedom of motion 
in their paretic extremities, was electricity, more especially the 
cutaneous faradization with the brush on the neck as well as 
up and down the limbs. If the constant current is used it 
ought especially to be applied to the muscles innervated by the 
ulnar and median nerves. By repeated closing and opening 
of the current muscular contractions should be elicited. 

21 



CHAPTER II. 

THE INFLAMMATIONS OF THE SOFT SPINAL MENINGES, 

Leptomeningitis Spinalis. 

The soft membranes are rarely ever by themselves the seat 
of inflammation, whether of an acute or a chronic type. Such, 
however, may exceptionally occur as the result of traumatism, 
of overexertion, carrying heavy loads, or as a consequence of 
exposure to cold after sleeping on the damp ground in camping 
out, etc. (Braun, cf. lit.). But in the greater number of cases 
we have to do with the extension of an inflammatory process 
of an infectious nature, as in epidemic cerebro-spinal menin- 
gitis, or in tubercular meningitis, both diseases which affect 
the membranes of the brain as well as those of the spinal cord. 
That there are instances of meningitis secondary to other dis- 
eases, and under what circumstances they occur, we have 
already stated above. Here we only wish to draw attention 
to its connection with acute articular rheumatism, of which 
Krabbel (Inaugural Dissertation, Bonn, 1887) has reported an 
instance. 

Pathological Anatomy. — Pathologically the acute spinal 
meningitis is divided into three stages. The first is character- 
ized by a diffuse reddening and swelling of the meninges, more 
especially of the pia; the second by the appearance of a puru- 
lent or fibrino-purulent exudation upon this membrane. This 
may occur gradually, and may vary considerably in extent ; it 
may be found over the whole length of the pia (always more 
on the posterior surface), or may be confined to circumscribed 
areas. In the third stage the pus becomes reabsorbed and 
thickening of the pia with the formation of adhesions between 
it and the dura takes place. 

That the nerve roots also participate in the inflammation is 
evident from the hyperemia of their blood-vessels, the infiltra- 
tion of the interstitial connective tissue, and the eventual de- 
322 



INFLAMMATIONS OF THE SOFT SPINAL MENINGES. 323 

generation of the medullated nerve fibres. If we remember 
the many processes by which the pia is united with the spinal 
cord itself, it is not surprising that the latter is implicated. 
On cross-section it looks in places injected, cedematous, and 
is seen to bulge ; on the other hand, there are undoubtedly 
instances in which the cord does not take part in the inflam- 
mation. 

The chronic form, which seems very rarely to occur pri- 
marily, and then only after the protracted abuse of alcohol, is 
usually preceded by the acute disease or is secondary to differ- 
ent spinal lesions or various affections of the vertebras. The 
pathological changes occurring here can well be compared 
with those of the acute form. In this condition we find thick- 
ening and opacity of the tissue, masses of newly formed con- 
nective tissue, and adhesions to the dura. There is turbidity 
of the spinal fluid, which is abnormally increased, and some- 
times abnormal formation of pigment. The brownish-red and 
black specks often seen are to be looked upon as the remains 
of previous haemorrhages, in which the coloring matter of the 
blood has undergone changes (Eichhorst). In consequence of 
the extraordinary development of the processes of the pia, this 
membrane adheres very firmly to the cord, so' that it can not 
be stripped off without loss of substance of the cord. Here, 
again, the nerve roots are implicated, as is evident from their 
changed appearance. They look flattened and atrophic. 

Symptoms. — In the acute form pain undoubtedly plays the 
principal part. Even in the initial stage, which does not differ 
from that of other acute diseases in most of the symptoms 
(chill, general malaise, loss of appetite, disturbed sleep, ele- 
vation of temperature), the pain along the spinal column is 
very marked. The patients are constantly troubled with it in 
whatever position they may lie, although it is especially sharp 
on any attempt to move or to sit up in bed. At the same time 
they feel an unwonted stiffness in the muscles of the back, and 
have difficulty on motion. On careful examination of the back 
we find that, although the spinous processes of the vertebrae 
are tender on pressure, and by the slightest tap or by the 
touch of a hot sponge pain is evoked, this is in no way com- 
parable to that felt by the patient without any extraneous in- 
terference. This persists obstinately, and usually in the further 
course of the disease may radiate into the arms and legs, 
owing, of course, to the implication of the nerve roots. The 



324 



DISEASES OF THE SPINAL MENINGES. 



same factor also accounts for the different hyperesthesias of 
the skin, the girdle sensation, the muscular pains, etc. Rigid- 
ity of the neck is only observed if the process has attacked the 
cervical portion. If the spinal cord itself becomes implicated, 
spinal symptoms, bladder disturbances, increased reflexes, and 
extensive sensory disturbances make their appearance. All 
these symptoms may persist unchanged for weeks, the patient 
feeling very badly and complaining of constant violent pain. 
If the disease take a favorable turn the pains gradually abate 
and the patient gets relief ; but, on the other hand, the symp- 
toms of irritation may give place to those of paralysis, and as 
anatomical changes go on in the nerve roots (degeneration, 
atrophy), we have analgesias and anaesthesias, the muscles be- 
come more and more incapable of performing their functions, 
they undergo marked atrophy, and on electrical examination 
distinct reaction of degeneration is found. There is direct 
danger to life (i) if the process extends upward to the medulla 
oblongata ; in that case death may occur in a few days ; (2) if 
owing to an extensive myelitis, bed-sores develop which lead 
to the utter exhaustion of the patient. Recovery may be com- 
plete or incomplete ; in the latter case pareses, paresthesias, 
and bladder disturbances are left behind as the result of irrepa- 
rable anatomical changes. 

The symptoms of the chronic do not differ much from those 
of the acute form. The pains, however, are occasionally less 
pronounced. They vary with regard to their violence and seat ; 
sometimes they are most marked high up between the shoulder 
blades, sometimes lower down in the back, so as to interfere 
more or less completely with stooping ; not rarely they are 
found to radiate toward the front of the thorax, sometimes on 
one, sometimes on both sides. Even slighter degrees of pain 
are sufficient to seriously interfere with the occupation of the 
patient, especially, of course, if the arms or legs, or what is, 
however, rather rare, all four extremities are implicated. Sen- 
sory changes are found in both the acute and the chronic form ; 
an implication of the cord itself leads to the same symptoms 
of irritation or paralysis which we have before mentioned. 
The disease may drag out its course through a number of 
years and still there may follow a relative recovery ; complete 
recovery I have never seen. 

Diagnosis. — To make a correct diagnosis of this disease 
much experience and carefulness is necessary. Acute spinal 



INFLAMMATIONS OF THF SOFT SPINAL MENINGES. 



325 



meningitis may be mistaken for muscular rheumatism and lum- 
bago, the chronic form for what was formerly called spinal 
irritation and cord diseases. A differentiation from the former 
may be facilitated by an examination of the spinous processes 
for tenderness on tapping or touching with a hot sponge. In 
simple muscular rheumatism the spinous processes are not sen- 
sitive, whereas the different muscles are found to be tender if 
pressed or kneaded. Lumbago pains are recognized by their 
greater severity, their frequent change in locality, and their 
lesser persistency. Spinal irritation should only be diagnos- 
ticated in very anaemic hysterical individuals ; and the further 
course and final outcome of the disease will guard us against 
the assumption of the existence of a cord disease, for, if this be 
present, the issue is always unfavorable. 

Treatment. — With reference to treatment, little is to be 
added to what has been said on page 321. Here, too, local 
measures — counter-irritation, etc. — must first be tried, and in 
case they should be found of no avail, prolonged tepid baths 
(93 Fahr., for from half an hour to an hour and a half) should 
be Substituted. Electricity should also be used in the form of 
the faradic brush applied over the painful muscles. Gentle 
massage, if practiced by a competent person, is strongly to be 
recommended, and ought to be continued for a long time. 
The administration of iodide of potassium, for which no indi- 
cation whatever exists, is to be condemned. 



CHAPTER III. 

HEMORRHAGE INTO THE SPINAL MEMBRANES MENINGEAL APOPLEXY 

PACHYMENINGITIS INTERNA HEMORRHAGICA. 

The vessels nourishing the spinal meninges are the anterior and 
posterior spinal arteries, arising from the vertebral artery, which in 
its turn comes off from the subclavian. They join with a succession 
of small branches which enter the spinal canal through the interver- 
tebral foramina and form median vessels, which run in front and 
behind the cord along the longitudinal fissure, having numerous hori- 
zontal anastomoses. Both of these arteries send constantly fine 
horizontal twigs into the substance of the cord, while others are dis- 
tributed to the pia. The capillary network is decidedly denser in 
the gray than in the white matter. 

The occurrence of a haemorrhage between the membranes 
of the spinal cord (" intrameningeal "), or between the dura 
and the bony vertebral canal (" extrameningeal "), is, on the 
whole, very rare. If one of these two forms occurs more fre- 
quently than the other, it is the latter, the extrameningeal, the 
so-called apoplexia epiduralis, so named because the blood es- 
capes into the epidural space. The haemorrhages between the 
dura and the arachnoid — apoplexia subduralis — and those be- 
tween the arachnoid and the pia — apoplexia subarachnoidalis — 
which break into the space filled with the cerebrospinal fluid, 
are much more uncommon. If we find on the inside of the 
dura encapsulated foci of variable size which contain products 
of decomposition, hasmatoidin crystals, detritus, etc., then we 
speak of a pachymeningitis interna hemorrhagica. The loose 
blood coagula may be found of such a size that they compress 
the cord and the nerve roots. On the other hand, there may 
be nothing more than punctiform extravasations of blood, in 
the neighborhood of which the vessels of the dura appear more 
than usually full. That these coagula are to a certain extent 
326 



MEN INGE A L AP OP LEX Y. 



327 



capable of being absorbed, and that they do not necessarily 
irreparably damage the cord and the nerve roots, is proved by 
the cases which take a favorable course. 

^Etiology. — With reference to the aetiology, it may be said 
that such haemorrhages may be evoked by overexertion. They 
occur by preference in men, and more especially in laborers 
who do hard work, such as carrying heavy loads, and who 
drink a great deal of alcohol. They may also follow traumatic 
influences, either direct injury to the bodies of the vertebrae 
or severe concussions affecting the whole body, such as one 
might receive, for instance, in a collision between two railroad 
trains, in which case symptoms arise which simulate very 
much the clinical appearances of railway spine, which we shall 
describe later. Secondary meningeal apoplexies occur in the 
course of infectious diseases — scarlet fever, small-pox, typhoid 
fever, etc. Also in epileptics they are not rare, and, according 
to Hasse, are often associated with heart hypertrophy. 

Symptoms. — The symptoms very closely resemble those of 
spinal meningitis, only that the onset is always very sudden — 
" apoplectiform." A person in perfect health may feel sud- 
denly a violent circumscribed pain in the back which differs in 
degree and extent in different cases, and which if the haemor- 
rhage is extensive may in a few hours give place to complete 
paralysis of the legs (more rarely of the arms). In milder 
cases, while the pains gradually abate, sensory disturbances, 
paraesthesias and anaesthesias, gradually develop, also slight 
motor disorders, weakness in the muscles of the extremities, 
sometimes also signs of motor irritation — trembling, twitching, 
etc. The main characteristic of a meningeal haemorrhage 
which is purely spinal is the complete freedom from disturb- 
ances of consciousness. The course and the duration of the 
disease depend upon the extent of the haemorrhage and its 
capability of being absorbed. It is necessary to have seen, 
carefully studied, and analyzed several cases of this nature in 
order to properly understand and correctly recognize a new 
instance. The implication of the spinal cord itself necessarily 
gives rise to what are known as " spinal symptoms " (increased 
reflexes, bladder disturbances, persistent paralyses), as we have 
repeatedly stated. 

Diagnosis. — The diagnosis is easy in the cases with charac- 
teristic onset if we are satisfied with the diagnosis of " menin- 
geal apoplexy," whereas it is very difficult, nay, often impossi- 



328 DISEASES OF THE SPINAL MENINGES. 

ble, to determine the exact kind, whether it is epidural or sub- 
dural. Again, to determine its situation is comparatively easy 
if we remember that in affections of the lumbar cord the legs, 
bladder, and the rectum mainly suffer, whereas affections of 
the dorsal region give rise to symptoms of irritation in the dis- 
tribution of the intercostal nerves, and those of the cervical 
portion to motor and sensory disturbances in the upper ex- 
tremities. If the seat be still higher up — in the medulla ob- 
longata — bulbar symptoms, disorders of respiration and deglu- 
tition, will not be absent, and the case will be fatal in a short 
time. 

Prognosis. — The prognosis depends upon the extent of the 
haemorrhage. Cases with a favorable outcome have repeatedly 
been observed. Implication of the cord and the nerve roots 
makes the prognosis more unfavorable. 

Treatment. — In the treatment, our first duty in a recent 
case should be to procure absolute rest in bed and apply ice 
over the supposed seat of the trouble, to arrest the haemor- 
rhage, if possible, or to prevent the return of it. If the irrita- 
tion seems to be localized, local bleeding may be indicated. 
The further treatment is the same as in acute meningitis. 

Tumors of the spinal meninges are of no practical impor- 
tance, because they can never be diagnosticated with certainty. 
Although we know well from the report of autopsies that just 
as in the cerebral we may in the spinal meninges find psam- 
momata, sarcomata, myxomata, gummata, carcinomata, etc., and 
that their seat may be epidural, subdural, and subarachnoidal, 
we are never able to recognize definitely from the symptoms 
observed during life either the nature or the seat of a tumor in 
the meninges of the cord. The reason is very simple. The 
tumors, as long as they are very small, produce no symptoms, 
and, if they grow, give rise to symptoms which depend upon 
the compression of the cord and the nerve roots and can not 
be distinguished from those produced by pachymeningitis and 
leptomeningitis spinalis. They consist, therefore, of signs of 
motor and sensory irritation and later of paralysis, which vary 
according to the seat of the tumor. If, for instance, only one 
half of the cord is compressed, we may have a clinical picture 
which resembles that of a unilateral lesion of the spinal cord, 
viz., paralysis and hypersesthesia on the side of the compression, 
anaesthesia on the intact side. A case of this kind has been re- 



TUMORS OF THE SPINAL MENINGES. 



329 



ported by Charcot (cf. lit.). Innumerable variations are pos- 
sible, according to the size and seat of the tumor, and the less 
we are able to fully diagnosticate the case during life the more 
important and instructive it will be to examine and describe as 
carefully as possible what is found at the autopsy. 

LITERATURE. 

Braun. Bemerkungen iiber die Meningitis spinalis, besonders nach Feldziigen, 

bei Officieren. Deutsche militararztl. Zeitschr., 1872, 1, 3, 4, p. 116. 
Leyden. Klinik der Ruckenmarkskrankheiten. Berlin, 1874, 1, pp. 443 et seq. 
Charcot. Klinische Vortrage iiber Krankheiten des Nervensystems. German 

by Fetzer, Stuttgart, 1878, ii, 83 et seq. 
Braubach. Arch. f. Psych, und Nervenkr., 1884, xv, 3, 489. (Lipoma of the 

Spinal Meninges.) 
Hirtz. De la pachymeningite cervicale hypertrophique curable. Arch, gener., 

Juin, 1886, p. 641. 
Raymond. Des differentes formes de lepto-myelites tuberculeuses. Revue de 

med., March, 1886, vi, 3. 
Senator. Ueber einige Falle von epidemischer Cerebrospinalmeningitis, etc. 

Charite-Annalen, 1886, xi, p. 288. 
Henoch. Zur Pathologie der Cerebrospinalmeningitis. Ibid., 1886, p. 525. 
Weichselbaum. Fortschritte der Med., 1887, v, 19. 
Eichhorst. Handbuch der speciellen Pathologie und Therapie, 3. Auflage, 

Wien und Leipzig, 1887, iii, 266 et seq. 
Goldschmidt. Centralbl. f. Bacteriologie und Parasitenkunde, 1887, ii, 22, p. 

. 649. (The Diplococcus Intracellularis Meningitidis.) 
Maguire. A Case of Idiopathic Suppuration of the Spinal Dura Mater. Lancet, 

July 7, 1887, p. 9. 
Cramer. Ueber multiple Angiosarcome der Pia mater spinalis mit hyaliner De- 
generation. Inaug.-Dissert., Marburg, 1889. 
Vogt. Ueber Meningitis spinalis. Deutsche med. Wochenschr., 1889, 38. 

(From Jiirgensen's Clinic.) 



PART II. 

DISEASES OF THE SPINAL NERVES. 

The nerves of the spinal cord, which are called spinal or periph- 
eral nerves, arise, as is well known, by an anterior smaller, and a 
posterior larger root. These are flat bundles of fibres, loosely sur- 
rounded by the arachnoid, which pass into the intervertebral foram- 
ina, where the posterior roots form a swelling, the ganglion inter- 
vertebrale, and emerge from the spinal canal, the two roots having 
united to form a common round trunk. This again divides after its 
exit from the canal into two branches, an anterior and a posterior. 
The anterior, usually the larger, forms numerous anastomoses with 
the branches above and below it, the so-called ansae, which are col- 
lectively called plexuses. The posterior, smaller nerves, pass back- 
ward between the transverse processes of the vertebrae, and are 
distributed to the muscles and the skin of the back. 

Of the thirty-one pairs of spinal nerves, there are eight cervical, 
twelve dorsal, five lumbar, five sacral, and one coccygeal. The pos- 
terior as well as the anterior branches contain fibres from both roots. 
The anterior roots are motor (Charles Bell, 1811). They supply, 
besides all the muscles of the trunk and extremities, the unstriped 
muscles of the internal organs and the unstriped muscles of the ves- 
sels. The posterior roots are sensory, but we should keep in mind 
that the anterior most probably contain, besides the motor, also tro- 
phic and secretory, and the posterior roots, besides the sensory, also 
fibres for the reflexes (cf. also Sass, Deutsche Med.-Ztg., 1890, 12). 

The peripheral nerves, just as the cranial, may be affected 
independently or secondarily, and as the result of some pri- 
mary disease in other parts. In cases of the first class over- 
strain plays an important role, often also, as we have seen to 
be the case in diseases of the cranial nerves, exposure to cold 
and traumatism, while in those of the second class a great many 
factors come in, more especially infections, intoxications, and 
general cachexias ; of these we shall speak when we treat of 
the individual nerves. 
330 



DISEASES OF THE PERIPHERAL NERVES. 331 

If we inquire into the anatomical character of the disease 
we shall in many instances have to admit that we are unable 
to find any anatomical changes whatever in the affected nerves. 
This is true in many cases of mild neuralgias, but also in some 
of the severe, even of the severest, types. The examination of 
pieces of the trigeminus, for instance, which were cut out 
where a resection had been made on account of intolerable 
pain has by no means always revealed appreciable changes in 
the nerve ; on the contrary, this has on microscopical, as well 
as on macroscopical, examination repeatedly been found to be 
absolutely normal. In other instances, however, an inflamma- 
tion — i. e., a neuritis — could be demonstrated as having been 
the cause of the trouble. In such cases there is seen in the 
acute stage an exudation in the interstitial tissue and an abun- 
dant infiltration of the same with round cells, a condition which 
gives rise to a swollen and cedematous appearance of the nerve 
(" purulent neuritis "). If this inflammation continues for some 
time the process goes on to degeneration, under the influence 
of which a part of the myeline sheath is destroyed and com- 
pound granular corpuscles are formed. The axis cylinders 
generally remain for some time intact. In some bundles there 
may be found nerve fibres completely atrophied, while the 
sheath is somewhat thickened and irregularly contracted, pre- 
senting a wavy outline. This increase and condensation of 
the connective tissue makes the nerve look more and more 
like a cord of connective tissue, which is thinner or thicker 
than normal according to the amount of the newly formed 
tissue ; sometimes, also, it is in places irregularly thickened 
(neuritis nodosa). The pigment deposits found have to be 
looked upon as the remains of previous haemorrhages. Even 
after extensive destruction of the nerve fibres by the connect- 
ive tissue, regeneration is to a certain degree possible, as the 
peripheral nerves possess this power to a considerable extent, 
a point which is of importance for the prognosis. According 
as the advance of the process is centrifugal or centripetal we 
speak of a descending or an ascending neuritis. A neuritis 
migrans has also been described. If the process occur simul- 
taneously at different places, we speak of a multiple or a dis- 
seminated neuritis (Leyden, Roth). From the researches of 
Scheube we should be led to regard the so-called beri-beri, or 
kak-ke, a disease which occurs epidemicallv in Japan, as a 
multiple neuritis. In very chronic cases the inflammatory 



332 DISEASES OF THE SPINAL NERVES. 

changes in the connective tissue are so slight in comparison to 
the degenerative process in the nerve fibres that it is prefera- 
ble to speak in those cases (as Striimpell has proposed) of a 
"primary chronic degenerative atrophy of the nerves," instead 
of a neuritis. 

The symptoms of neuritis, of course, vary according to the 
position and the function of the affected nerve, as we shall show 
in the following pages. The symptomatology of the primary 
multiple neuritis par excellence we shall describe later. 

The peripheral nerves may also be the seat of neoplasms, 
which, when developing in them, usually start from the connect- 
ive tissue. Only rarely do they consist of newly formed nerve 
fibres, and deserve properly to be called neuromata ; much 
more frequently they are fibromata, which may be found as 
solitary or as multiple new growths, and which not uncom- 
monly may give rise to thickenings and nodular swellings, 
which can be easily demonstrated and felt on the nerves. Ex- 
tensive tumors, where numerous nerve trunks are united by 
connective tissue into a compact mass, the so-called plexiform 
neuro-fibromata, are rare. Malignant neoplasms, carcinomata, 
and sarcomata of the peripheral nerves are sometimes met 
with. That here, also, the symptoms depend on the seat of 
the new growth is self-evident (cf. Krause on Malignant Neuro- 
mata and the Occurrence of Nerve Fibres in them. Volk- 
mann'sche Sammlung klin. Vortrage, 293, 294, 1887. Deutsche 
Med.-Zeitung, 1888, No. 15). 

We shall first speak of the affections of the motor and the 
sensory nerves which innervate the muscles of the extremities 
and the trunk, and certain internal organs which are not con- 
nected with the cranial nerves, and after that we shall turn our 
attention to the trophic, the vaso-motor, and the secretory 
fibres as far as our scanty knowledge on these points will 
allow. An appendix will be devoted to the primary affections 
of the muscles supplied by the spinal nerves. 

A. Diseases of the Motor and Sensory Nerves. 
/. Diseases of the Cervical Nerves. 

Of the four upper (smaller) cervical nerves, the first, which is 
called the suboccipital, emerges between the occipital bone and the 
atlas. The anterior branches of these four form the plexus known 
as the plexus cervicalis, which is situated opposite the correspond- 



THE CERVICAL PLEXUS. 



333 



ing vertebrae. From this plexus come, besides the muscular branches 
to the scalenus, the longus colli, etc., the phrenic, which is formed 
chiefly by the fourth cervical nerve, and which for the most part is 




Fig- 93-— Diagrammatic Outline of the Cervical and Brachial Plexuses. (After 
Schwalbe.) CI-VIII, roots of the cervical nerves. DI-III, roots of the first three 
dorsal nerves, pp, posterior branches— p 2 , of the second, p z , of the third cervical 
nerve, i, anterior branch of the first cervical nerve and loop of union with the second. 
2, small occipital nerve. 3, great auricular nerve. 3, superficial cervical nerve. yi, 
communicating branches to the descendens noni from the second and third. 3S, com- 
municating to the accessorius from the third and fourth nerves. 4, supraclavicular nerves. 
4,, phrenic nerve. Brachial plexus : V r VIII t , and D ,, the five roots of the brachial 
plexus. 5, rhomboid nerve. 5, suprascapular. $ ti , posterior thoracic. 6, nerve to the 
subclavius muscle. 7, 7, inner and outer anterior thoracic nerves. 8, 8,, 8 (/ , subscapu- 
lar nerves. MC, musculo-cutaneous. M, median. U, ulnar. MS, musculo-spiral. ic, 
internal cutaneous. TV, nerve of Wrisberg. c, circumflex, i, i, intercostal nerves. -ih, 
intercosto-humeral nerve. 



a motor nerve, the superficial cervical, the auricularis magnus, the 
occipitalis minor, and several communicating branches to the upper 
cervical ganglion and the gangliform plexus of the vagus (Fig. 93). 
The anterior branches of the four lower (the stouter) cervical 



334 DISEASES OF THE SPINAL NERVES. 

nerves, after they have passed between the anterior and middle 
scalenus and have reached the supraclavicular fossa, form, in con- 
junction with the anterior branch of the first dorsal nerve, the so- 
called brachial or subclavian plexus, which may be divided into a 
smaller or supraclavicular portion situated above, and a larger infra- 
clavicular portion situated below, the clavicle. From the former are 
given off, besides the suprascapular, only the three subscapular 
nerves, the anterior and posterior thoracics, and the rhomboid nerve. 
The larger portion, which has also been called the axillary plexus, 
furnishes the large nerves which supply the entire upper extremity, 
the circumflex (axillaris), the median, the ulnar, the muscuio-spiral 
(radial) and cutaneous branches, namely, the nerve of Wrisberg, the 
internal, and the (longest) external or musculo-cutaneous nerve, 
which has also been called perforans Gasseri (cf. Knie, Beitrag zur 
Frage der Localisation der motorischen Fasern im Plexus brachia- 
lis, Internat. klin. Rundschau, 1889, 14). 

Just as we have seen in speaking of the cranial motor 
nerves — e. g., the oculo-motorius, the abducens, and the facial 
— the motor disturbances of the spinal nerves may be of a 
paralytic or of an irritative character. In the former case the 
mobility of the muscles supplied by the affected nerve is di- 
minished (paresis) or completely lost (paralysis). In the latter 
we have symptoms of motor irritation which are not under the 
control of the will, the so-called spasms. These consist either 
of transient muscular contractions or of a lasting state of spas- 
modic contraction of one or of several muscles. The former 
we call clonic, the latter tonic, spasm. 

On the whole, paralytic symptoms are much more common 
in the distribution of these plexuses than sy 7 mptoms of irritation. 

In the sensory disturbances we can equally distinguish 
paralytic from irritative conditions, the former giving rise to 
anaesthesia, the latter to hyperaesthesia. The anaesthesia is 
characterized by the fact that external (mechanical, chemical, 
or thermic) stimuli are either not perceived at all or with di- 
minished acuteness, whereas in hyperaesthesia, on the contrary, 
even very weak stimuli are felt to be abnormally strong and 
unpleasant. The latter condition is usually attended with 
symptoms of sensory irritation, manifested by pronounced 
pains or by paraesthesias — that is, abnormal sensations of prick- 
ing, formication, numbness, or a " furry feeling." 

The affections of the sensory fibres of the spinal nerves 
manifest themselves chiefly by symptoms of irritation. They 



THE BRACHIAL PLEXUS. 



335 



are always associated with more or less pain and are called 
neuralgias. That these also occur in the cranial nerves has 
already been stated, and the trigeminal neuralgia (cf. page 68) 
may be taken as a type of them. The neuralgic pains are 
usually very violent, but are rarely or never constant. They 
appear periodically and follow fairly accurately the distribu- 
tion of the affected nerve. The diagnosis is rarely difficult. 
Peripheral anaesthesias — that is, such as are only due to affec- 
tions of the peripheral nerves or their end organs — are, as we 
said, rare. 



CHAPTER I. 

LESIONS OF THE CERVICAL PLEXUS. 

The cervical plexus is, on the whole, much less frequently 
affected with motor disturbances than the brachial. Among 
the nerves belonging to it, it is the phrenic more especially 
which may present symptoms of paralysis or of irritation ; yet 
neither paralysis nor spasm of the diaphragm due to disease of 
the phrenic is of any great practical importance, since such an 
affection scarcely ever occurs by itself, but is much more often 
met with only when associated with other diseases. Paralysis, 
for instance, is observed in the course of progressive muscular 
atrophy, in hysteria, probably also in lead poisoning. Trauma- 
tism or mechanical compression produced by tumors or ab- 
scesses in the neck may be the cause. Recently it has also 
been observed in tabes (Berliner klin. Wochenschr., 1893, 
xvi). Among the signs of paralysis of the diaphragm there is 
one which is very conspicuous, namely, the faulty expansion 
in the epigastric region during inspiration. Instead of becom- 
ing prominent, as is the case in the normal condition, the epi- 
gastrium is drawn in, and when we lay our hand on it we can 
feel that the diaphragm does not descend. If only one of the 
phrenic nerves is thus affected this phenomenon is present only 
on one side, while the other half of the diaphragm performs 
its function properly. Besides this, hardly any other symp- 
toms are observed in uncomplicated cases if the patient remains 
at rest, whereas if he exerts himself a distinct dyspnoea and an 
increase in the frequency of the respirations become apparent. 
The obstinate constipation which such patients complain of 
can well be understood if we remember the part which the 
diaphragm takes in the abdominal pressure. 

Spasm of the diaphragm, at least the tonic form of it, is not 
more common than paralysis. Patients affected with this suffer 
from great shortness of breath and quickly become cyanosed. 
336 



LESIONS OF THE CERVICAL PLEXUS. 



337 



The markedly prominent epigastrium remains with the dia- 
phragm immobile and is tender to the touch, and only the 
upper part of the thorax shows shallow respiratory movements. 
In some cases of tetanus, tonic spasm of the diaphragm seems 
to be the cause of death. It occurs almost never by itself with- 
out some accompanying or underlying affection, except in hys- 
terical persons. On the other hand, the clonic form of the 
spasm, the so-called hiccough (singultus), is extremely common. 
Everybody is familiar with the short clonic movements of the 
diaphragm, which are accompanied by inspiratory sounds and 
which vary in frequency and severity, occurring sometimes in 
such rapid succession that eighty or even a hundred may be 
counted in one minute. Severe protracted hiccough may be- 
come very troublesome, indeed, even dangerous, if sleep is for 
a long time seriously interfered with. This is, however, only 
the case if singultus occurs as a symptom in the course of 
other diseases — e. g., in apoplexy, in peritonitis, in chronic gas- 
tric catarrh, etc. Even when it appears as a reflex neurosis — 
e. g., in the course of a chronic gastro-enteritis — it may cause 
a great deal of trouble to the patient (Dehio, Berliner klin. 
Wochenschrift, 1889, 22). As a rule it is arrested without any 
interference on the part of the physician by popular methods, 
such as holding the breath, closing the glottis and then at- 
tempting an expiration. 

Therapeutics is almost powerless in the face of affections 
of the motor fibres of the phrenic. In paralysis, electrical 
stimulation of the nerve, in the (tonic) spasm, chloroform and 
morphine, have been recommended ; yet these measures are by 
no means reliable. 

The sensory fibres which the phrenic takes up in its course, 
and which are distributed to the pleura, the pericardium, and 
the peritonaeum, may also be affected. Neuralgia of the 
phrenic is rare, or perhaps we had better say is undoubtedly 
but rarely recognized. The pains, starting at the base of the 
thorax at the points corresponding to the insertions of the 
diaphragm and radiating in all directions, are taken for rheu- 
matism of the chest muscles or intercostal neuralgia, and it is 
only in cases in which the pain is felt directly over the scalenus 
anticus and corresponds to the course of the nerve that the 
diagnosis is made correctly. Valleix's painful points can occa- 
sionally be demonstrated on the spinous processes of the 
upper cervical vertebrae and at the points of insertion of the 
22 



338 DISEASES OF THE SPINAL NERVES. 

diaphragm. Respiration is interfered with only when the mo- 
bility of the diaphragm is at the same time impaired. 

The aetiology of the disease is obscure ; more especially are 
we ignorant of the conditions under which it may occur in- 
dependently. It seems not to be a rare accompaniment of 
Graves' disease, of angina pectoris, and of sclerosis of the 
coronary arteries. 

Another apparently more important neuralgia in the region 
of the cervical plexus is the occipital or cervico-occipital neu- 
ralgia, which attacks by preference the occipitalis major, but 
also the minor, further the auricularis magnus, the subcutaneus 
colli and the tympanic nerve or plexus, which belong to the 
glosso-pharyngeal nerve (Jacobson's anastomosis). The pa- 
tients complain of pain in the whole occipital region, in the 
neck, often, too, in the ears. Much more rarely the pain radi- 
ates in a forward direction to the cheek and the lower jaw. 
The so-called otalgia nervosa may give rise to such excruciat- 
ting pains that the patient's consciousness may become clouded 
(Gompertz, Centralblatt f. d. ges. Therap., 1890, Heft 5), and 
very severe pains may also be produced by an affection of the 
tympanic plexus. In such cases it is important to examine for 
ulceration around a tooth or in the larynx. Such patients 
dread every motion of the head, and carefully avoid every 
cause for laughing, as this, as well as sneezing, chewing, and so 
forth, is liable to bring on an attack. The consequent rigid 
position of the neck is quite characteristic for this form of neu- 
ralgia. Painful points can sometimes be found at the exit of 
the occipitalis major — that is, about halfway between the mas- 
toid process and the spinous processes of the cervical vertebrae. 
Where they are absent the disease may be confounded with 
torticollis rheumatica ; yet such a mistake may be avoided by 
remembering that the neuralgia is not constant, but character- 
ized by intervals of perfect ease. 

The course of an occipital neuralgia is often tedious, but 
on the whole it is not unfavorable, and complete cures are 
not rare. The prognosis is bad only when there exists some 
organic lesion of the nerve, caused, for instance, by disease of 
the cervical vertebrae. If, as is usually the case, no definite 
cause can be found, energetic counter-irritation to the skin, 
local bleeding, galvanization, the application of moist or dry 
heat, or the use of antipyrin or phenacetin, will usually effect 
a cure or at least an improvement. Subcutaneous injections 



LESIONS OF THE CERVICAL PLEXUS. 



339 



of morphine we shall probably in most cases be able to dis- 
pense with. The removal of every deleterious cause should, 
of course, be insisted upon. Under certain circumstances the 
occupation has something- to do with it. Thus, I have found 
that the stevedores of the London docks, who carry extremely 
heavy weights on their backs which press upon the neck and 
the occiput, are frequently subject to occipital neuralgia (cf. 
Hirt, Krankheiten der Arbeiter, iv, 91). 



CHAPTER II. 

LESIONS OF THE BRACHIAL PLEXUS. 

The brachial plexus may be diseased in its supra- or infra- 
clavicular portion. The affections of the latter, undoubtedly 
the more frequent, are of greater practical importance than 
those of the former. 

Here, too, the motor disturbances are more prevalent, sen- 
sory disturbances in the region of the brachial plexus, espe- 
cially neuralgias, being decidedly exceptional. In a case re- 
ported by Stern (Berliner klin. Wochenschr., 1891, 46), the 
compression exerted by a bandage had produced an affection 
of the whole brachial plexus, with consequent arrest in growth 
and extensive atrophic paralysis. 

In the supraclavicular portion, the posterior thoracic — 
which, coming from the fifth and sixth cervical nerves, supplies 
the serratus magnus — is affected in an interesting and very 
striking manner. 

The so-called serratus paralysis is quite frequently due to 
the calling of the patient, as certain occupations seem particu- 
larly to predispose to it. If prolonged pressure is frequently 
exerted on the nerve — as, for instance, is the case in people 
who carry heavy loads on their shoulders, or if the shoulder 
muscles, especially the serratus, are overexerted, as happens, 
for instance, in mowing, in certain manipulations of tailors, 
shoemakers, etc. — the paralysis has been known to develop 
rapidly. Occasionally such aetiological factors are absent, and 
we are forced to fall back upon the still obscure influence of 
what is called " catching cold." 

The condition is quite characteristic whether the arm be in 
a state of motion or at rest. In the latter position the scapula 
appears elevated and approaches with its lower angle the ver- 
tebral column more than normally, the inner median margin 
having an oblique upward and outward direction. The cause 
340 



LESIONS OF THE BRACHIAL PLEXUS. 



341 



of this deformity is to be sought in the overaction of the an- 
tagonists — the rhomboids, the levator anguli scapulae, and the 
trapezius (Fig. 94). On moving the arm, the patient, we find, 
can raise it only to the horizontal position, owing to the ab- 
sence of the action of the serratus, which pushes the scapula for- 
ward. As soon as we produce artificially the action of this 




Fig. 94.— Case of Right-sided Serratus Paralysis in a Man Thirty-five Years 
of Age. Position of the scapula with the arm hanging down. (After Eichhorst.) 

muscle by fixing the shoulder blade and pushing it forward, 
complete elevation of the arm is possible. If the patient at- 
tempts this same motion himself the scapula is approached to 
the spinal column. If the arm is raised in front of the chest 
the inner edge of the scapula is elevated and stands off from 
the thorax in a winglike fashion, so that we are able to touch 
the inner surface of the bone (Fig. 95). Besides a moderate 



342 



DISEASES OE THE SPINAL NERVES. 



impairment in adduction, which somewhat interferes with the 
folding of the arms across the chest, there are no other abnormi- 
ties to be mentioned. Especially is it to be noted that there 
are no decided sensory changes to be perceived in a pure ser- 
ratus paralysis. As this affection is not rarely met with in the 
course of progressive muscular atrophy — sometimes this dis- 




Fig. 95. — The same Case with the Arms raised. 

ease begins with a serratus paralysis — it is not to be wondered 
at that the muscle at fault is sometimes found to be wasted. In 
the traumatic paralysis the atrophy comes on very late — many 
years after the traumatism. The muscle remains intact, elec- 
trical reactions are normal — reaction of degeneration being by 
no means always demonstrable — and yet there is no improve- 
ment. The prognosis, on the whole, is bad ; the disease even 



LESION'S OF THE BRACHIAL PLEXUS. 



343 



in the most favorable cases is of very long duration, and may 
last for weeks, months, or years. Often it is not curable at all, 
and the patient is, as it were, maimed for the rest of his days. 

Not too much hope ought to be placed in the electrical 
treatment, no matter in what form electricity be employed ; in 
grave cases, at least, such hopes are doomed to disappointment. 

The paralyses of the pectoralis major and minor (anterior 
thoracic nerves), of the rhomboidei and the levator anguli 
scapulas (muscular branches from the third, fourth, and fifth 
cervical nerves), of the latissimus dorsi, subscapularis, and 




Fig. 96.— Position of the Head in Spasm of the Splenius Capitis on the 

Right Side. 

teres major (subscapular nerves), finally, those of the supra- 
spinatus and infraspinatus (suprascapular nerve), have by them- 
selves no practical importance, although isolated affections of 
the last nerve have, of late especially, been repeatedly ob : 
served. Thus Bernhardt has reported an instance occurring 
after contusion of the shoulder joint (Erlenmeyer's Centralbl. 
f. Nervenheilk., 1889, 7) ; F. Schulze, a case in which the affec- 
tion was produced during birth (Arch. f. Gynac, 1888, 3); 
Sperling, one in which, after neuritis of the whole brachial 
plexus, an improvement took place in all branches except the 



344 



DISEASES OF THE SPINAL NERVES. 



suprascapular (Neurol. Centralblatt, 1890, 10); finally, Beuzler 
has reported a case in which he found atrophy of the muscles 
supplied by this nerve (Deutsche med. Wochenschrift, 1890, 51). 

Spasms of the muscles concerned here are also unusual. A 
characteristic position of the head is evoked by a unilateral 
spasm of the splenius capitis (Fig. 96). Bilateral spasm of the 
deep muscles of the neck produces a strong retraction of the 
head, while spasm of the rhomboids alters the position of the 
shoulder blades, etc. 

Of the nerves belonging to the infraclavicular portion of 
the brachial plexus none is so frequently the seat of disease as 
the continuation of the posterior trunk of the plexus, which 
becomes the musculo-spiral or radial nerve, and supplies the 
skin and the muscles of the extensor surface of the arm. 




Fi S- 97.— Musculo-spiral Paralysis. 



The musculo-spiral paralysis can better than any other form 
be recognized at a glance. A patient with paralysis of the 
extensors is unable on stretching out the arm to raise the hand, 
while lateral motion is difficult. Dorsal flexion, which is per- 
formed by the extensor carpi radialis and the extensor carpi 
ulnaris, is impossible, abduction and adduction difficult, the 
hand hangs down flaccidly (" wrist-drop," cf. Fig. 97), and 
when rested upon the table can not be raised. On a more 
careful examination it is noticed that the first phalanx of the 
flexed fingers can not be extended without assistance, but that 
if this phalanx is passively extended the patient can straighten 
out the others himself. The first condition is due to the paraly- 
sis of the extensors, which, as is well known, on the dorsal 



LESIONS OF THE BRACHIAL PLEXUS. 



345 



surface of the first phalanx pass into an aponeurosis ; the sec- 
ond to the preservation of the function of the interossei, which 
are supplied by the ulnar nerve. Since its extensors are also 
implicated, the thumb, of course, can not be actively extended, 
neither can it be abducted, because the muscles concerned are 
also paralyzed. Some interesting conditions will be found on 
examination of the forearm in extension and flexion. If, for 
instance, the forearm is extended and pronated, supination is 
impossible, because the supinator brevis is paralyzed. During 
flexion of the forearm, however, the biceps, which is intact, 
can perform supination without difficulty. If the forearm is 
in a position of supination it is easily flexed by the intact 
muscles, the biceps and the brachialis anticus, while if it is 
half pronated flexion is imperfect, owing to the paralysis of 
the supinator longus. The characteristic prominence formed 
by the belly of this muscle when the forearm is flexed is abso- 
lutely wanting. Any participation of the triceps in the paral- 
ysis is only observed if the lesion is high up (" crutch palsy "). 
Usually the injury is situated where the nerve turns over the 
humerus or lower down, in which case naturally the normal 
function of the triceps is not interfered with. Isolated paral- 
ysis of the triceps is very rare ; a case of this kind has been 
published by Oppenheim (Berlin, klin. Wochenschr., 1889, 44). 
The patient was a weaver, and the affection was regarded as 
having been due to his occupation. Permanent trophic dis- 
turbances, shown by pronounced wasting of the affected mus- 
cles, are rare in cases of pressure paralysis, while they are fre- 
quent in the paralysis developed as a consequence and in the 
course of lead poisoning. 

The flexors, otherwise perfectly healthy, also become weak- 
ened, because their points of insertion are approximated to the 
points of origin more closely than under normal conditions, on 
account of the constant drooping of the hand, and hence the 
interference with motion is aggravated. The patient can 
hardly use the hand at all ; he is unable to take hold of any- 
thing, the finer manipulations necessary for writing, drawing, 
etc., are impossible, and in the majority of cases he is unfit for 
work or for making a living during the whole course of the 
disease. 

Sensory changes are rarely sufficiently marked to add much 
to his troubles. Sometimes paresthesias may be complained 
of — a feeling of cold, numbness, formication, and the like ; 



346 DISEASES OF THE SPINAL NERVES. 

sometimes, also, there is a distinct decrease of sensibility, so 
that zones of anaesthesia can be made out. Pagenstecher has 
published the results of his study of these conditions in an 
article (Arch. f. Psych., 1892, xxiii, 3, p. 838), in which will also 
be found a careful collection of references to the literature. 
On the other hand, a source of great annoyance is found in 
the peculiar painless swellings of the extensor tendons on the 
back of the hand. These node-like swellings have been de- 
scribed by Gubler as tenosynovitis hypertrophica, and are to 
be attributed to mechanical influences acting injuriously on the 
tendon. 

The duration and course of a musculo-spiral paralysis may 
vary greatly, and it is often hard to give an opinion on these 
points at the very onset of the affection. An electrical exami- 
nation, which reveals the reactions of the muscles and nerves 
to the faradic and galvanic current, is the only means by which 
we can arrive at an opinion as to the duration of the disease. 
The conditions are the same as those we described as existing 
in facial paralysis, and it suffices, therefore, to refer the reader 
to that chapter. But here again be it stated, a prognosis should 
never be given without a previous electrical examination of 
nerves and muscles. 

The aetiology of musculo-spiral paralysis is interesting from 
the fact that it is fairly well understood. While, as all con- 
fess, the cause of most nervous diseases is absolutely unknown, 
and we therefore are forced to fall back on uncertain explana- 
tions, such as exposure to cold, it seems, according to our 
present knowledge, that musculo-spiral paralysis always can 
be traced back to one or two kinds of causes, viz., mechanical 
or chemical. There are quite a number of lesions due to 
mechanical or traumatic causes. Frequently a man, when 
greatly fatigued, drunk, or exhausted, goes to sleep, using his 
arm, usually the left, as a support for his head ; the latter, 
pressing on the nerve in the lower third of the humerus, gives 
rise to an injury in a relatively short time, or the arm support- 
ing the head of the sleeper may press with its outer side 
against a chair or the like and a paralysis be the result. This 
is the so-called " sleep palsy." Next we have compression 
happening to the patient as a consequence of his daily occu- 
pation, due to pressure from ropes, handles of water-jars (as 
in the water-carriers' paralysis of Rennes), etc. ; sometimes 
in infants this paralysis occurs from too much compression 



LESIONS OF THE BRACHIAL PLEXUS. 



347 



on the arms by too tight swathing-clothes ; sometimes too 
tight plaster-of-Paris bandages have been the cause; and, 
finally, all direct injuries to the nerve — stab wounds, blows, 
gunshot wounds, and compression of the nerve by abnormal 
callus formation after fracture of the humerus — must also be 
mentioned. 

The lesions due to chemical causes may be the result of the 
action of certain poisons, among which lead deserves to be 
mentioned first. It is a fact no less remarkable than well au- 
thenticated, to which we shall again refer when speaking of 
lead poisoning in general, that this agent acts by preference 



M. extensor carpi radialis brevior 
M. extensor digitorum communis 
M. extensor minimi digiti 
M. extensor indicis 
M. abductor longus pollicis 
M. extensor brevis pollicis 
M. extensor longus 
pollicis 



Nervus muscirto-sjriralis 
M. brachialis anticus 
M. supinator longus 
M. extensor carpi 
radialis longior 
M. extensor carpi 
ulnaris 



M. triceps 
{external head) 

M. triceps 
(long head) 




Fig. 98.— Motor Points of the Musculo-spiral Nerve and the Muscles 

SUPPLIED BY IT. 



on the muscles which are supplied by the musculo-spiral nerve. 
This musculo-spiral paralysis, however, unlike the form which 
is produced by mechanical action, is not an independent dis- 
ease, but merely a symptom of a general intoxication. Accord- 
ing to the commonly received opinion (Leyden and others), the 
paralysis depends upon a degenerative atrophy of the motor 
peripheral nerve fibres, to which is often superadded a spinal 
affection. It differs in its clinical aspect from the mechanical 
lesion, inasmuch as the supinator longus and the triceps remain 
intact. Of late years several cases have been published where, 
after subcutaneous injections of ether into the extensor surface 
of the forearm for therapeutic purposes, a musculo-spiral pa- 
ralysis appeared (Falkenheim, Arnozan, Remak, H. Neumann, 



348 



DISEASES OF THE SPINAL NERVES. 



cf. lit.). In using the drug in this way this possibility ought to 
be thought of. 

In contradistinction to the frequency with which paralysis 
is found, signs of irritation in the distribution of the musculo- 




cl 




10me 
Fig. 99. Fig. 100. 

The Distribution of the Cutaneous Nerves of the Arm and Hand. (After 
Eichhorst. ) Fig. 99, volar surface of the upper extremity, isc, supraclavicular nerve. 
2ax, circumflex nerve. 2> cmc ^i internal cutaneous nerve. 4c/, external cutaneous nerve. 
$cm } cutaneous medius. 6me, median nerve. 7«, ulnar nerve. Fig. 100, isc, supra- 
clavicular nerve. 2ax, circumflex nerve. 2,cps, superior posterior cutaneous nerve. 
5Cpz, inferior posterior cutaneous nerve. 4cmd, internal cutaneous nerve. 6cm, median 
cutaneous nerve, jc/, external cutaneous nerve. Su, ulnar nerve, gra, musculo-spiral 
nerve, xome, median nerve. 

spiral nerve — that is, spasms — are extremely rare. They have 
been observed most often after manual overexertion — gymnas- 
tics, etc. (Hochhaus, Deutsches med. Wochenschr., 1886,47; 
Laqueur, xiv. Wanderversammlung der siidwestdeutschen Neu- 
rologen, Arch. f. Psych., 1889, xxi, 2, p. 660). 

In the treatment, electricity not only plays the chief, but the 
only role. From the motor points (Fig. 98) the muscles ought 
to be stimulated with the constant current, and, besides this, 



LESIONS OF THE BRACHIAL PLEXUS. 



349 



Imi 



frequent extensive applications of the faradic brush to the skin 
of the affected arm ought to be practiced. That the cause, if 
such should be present — for instance, pressure of crutches, of 
dislocated bones, etc. — ought to be removed, is self-evident. 
Heusner (Barmen) demonstrated before the Association of Natu- 
ralists in Halle (1891) an apparatus by means of which the 
action of the extensors is replaced by rubber cords ; this appa- 
ratus has proved to be satisfactory. The sensory disturbances 
which may be found in the distribution of the musculo-spiral 
we shall mention when considering cervico-brachial neuralgia. 
The mode of distribution of the cu- 
taneous nerves of the upper extrem- 
ity is illustrated in Figs. 99, 100, 10 1. 

The median and ulnar nerves supply 
together the innervation of the muscles 
and the skin of the inside of the fore- 
arm and the hand, the former innervating 
almost all the flexors of the forearm, the 
pronator radii teres, and the pronator 
quadratus, the flexor carpi radialis, the 
flexor sublimis digitorum, and a part of 
the profundus, leaving the flexor carpi 
ulnaris to the ulnar. Among the thenar 
muscles the median supplies the abduc- 
tor brevis, the opponens, the outer head 
of the flexor brevis, further, the first 
three lumbricales, while it again leaves 

to the ulnar, besides the one flexor mentioned, the antithenar, the 
adductor brevis pollicis, the deeper head of the flexor brevis pollicis, 
the fourth lumbricalis, and all the interossei. 

Both nerves have this in common : that they only rarely 
become affected by themselves, much more rarely than the 
musculo-spiral, and that they are, unlike the latter nerve, liable 
to disturbances not only in their motor but also in their sensory 
fibres. We shall have to speak, therefore, not only of paral- 
yses, but also of neuralgias. With regard to the aetiology, we 
may consider it as the rule, just as in musculo-spiral paralysis, 
that motor disturbances only occur as a consequence of me- 
chanical injury, provided there be no other disease present — 
e. g., progressive muscular atrophy and the like ; while neural- 
gias may appear under other circumstances — e. g., after acute 
diseases, after exposure to cold, sometimes also without any 




Fig. 101. — Distribution of the 
Sensory Nerves on the Back 
of the Fingers (Krausei. 
r, musculo-spiral nerve, u, ul- 
nar nerve, m, median nerve. 



35o 



DISEASES OF THE SPINAL NERVES. 



demonstrable cause. The ulnar paralysis may be caused by 
certain occupations, as Duchenne has already observed re- 
peatedly in workingmen who are obliged to press the elbow 






*§ 




s 

cis 
ollici 




5 


tor radv, 

digitorv 
dus 


« 

'& 

§ 
^ 


*e oo S fe» 


nus 

(or brevi 

ens polli 
brevis p 


■43 


s 

•1 




S 
J 




\edia\ 
bduc 
llicis 
ppon 
'exor 


"3 60 






**» 


^ <*> 




e a. 


N 


^~ *j 


N 



Fig. io2.— Motor Points of the Median Nerve and the Muscles supplied by it. 

firmly upon a hard surface. It is not a rare occurrence in 
those who have to use the ulnar side of the hand — hypothenar 
eminence — a great deal to strike certain instruments (cabi- 
net-makers, dyers, cobblers, etc.). 
V 




N. ulnaris 



M. flexor carpi ulnaris 

Fig. 103. 



^ M. Ivmbri- 

cales III et IV 

M. opponens min. dig. 

, M. flexor minimi digiti 

, M. abductor minimi digiti 

M. palmaris brevis 

Nervus ulnaris 



A pure median paralysis is chiefly characterized by the in- 
ability to pronate the forearm and to flex the hand, as we can 
easily understand from the anatomy of the parts. A very 
slight flexion of the hand toward the ulnar side is, however, 



LESIONS OF THE BRACHIAL PLEXUS. 



351 



rendered possible by the action of the intact flexor carpi ul- 
naris. The terminal phalanges can not be bent, but in the first 
phalanges, which are under the control of the interossei, this 
motion is not impaired. The part of the flexor profundus digi- 
torum which is supplied by the ulnar makes it possible for the 
patient to seize some objects with the third, fourth, and fifth 
fingers. The extended and adducted thumb, which lies in 
close apposition to the index finger, is almost useless. 

On the other hand, we find in ulnar paralysis that the 
thumb can not be pressed against the index finger on account 
of the paralysis of the adductor pollicis, that the terminal 
phalanges of the fingers can not be straightened, the first ones 
not flexed (paralysis of the interossei), and that the little finger 




M. interossei J-IY 



M. abductor minimi di 



Fig. 104. — Motor Points of the Ulnar Nerve. 



is almost wholly useless. With the median paralysis the ulnar 
form has this in common, that flexion at the wrist joint is greatly 
impaired. In the latter especially lateral movement toward 
the ulnar side is interfered with owing to the paralysis of the 
flexor ulnaris. Lastly, the difficulty which is experienced by 
the patient in spreading his fingers apart and bringing them 
together again, movements which are indeed almost impos- 
sible, greatly facilitate the diagnosis of ulnar paralysis, which, 
however, for that matter, is always simple. 

Muscular atrophies not uncommonly develop in both of 
these paralyses, but more frequently in the ulnar form. The 
interosseal spaces on the back of the hand become sunken in, 
and, if the wasting affects chiefly the interossei and the lumbri- 



352 



DISEASES OF THE SPINAL NERVES. 



cales, the hand assumes a peculiar appearance. It becomes 
not unlike a claw, since the healthy antagonists — the extensor 
digitorum communis and the flexor digitorum — produce a dor- 




Fig. 105. — Claw-hand. (After Duchenne.) 

sal flexion of the first phalanges and a complete palmar flexion 
of the second and third (cf. Fig. 105). This is called the " claw 
hand," the " main en griff e 11 of the French. 

Atrophy confined to the antithenar eminence I have repeat- 
edly observed in cabinet-makers. They themselves attribute 
it to the continued use of the plane. 

The affections of the sensory fibres of the median and ulnar 
nerves may either occur alone or be found associated with 
those of the motor fibres. In the latter case we have to con- 
tend with disturbances of sensibility, paresthesias, numbness, 



N. mtbsculo-cutaneus 

M. brachialis anticus 
M. biceps brachii 




Fig. 106.— Motor Points of the Musculo-cutaneous Nerve and the Muscles 

SUPPLIED BY IT. 



anaesthesia, and pains, sometimes quite well pronounced, which 
are most marked in the initial stage of the paralysis. In the 
former there are genuine neuralgias, acute, spontaneous, lanci- 



LESIOXS OF THE BRACHIAL PLEXUS. 



353 



nating pains which follow the course of the nerve and which 
are intensified by pressure upon it. Such pains are more fre- 
quently observed in the distribution of the median than in that 
of the ulnar, but they are not common in either of these re- 
gions. 1 have known them to occur occasionally after acute 
diseases, especially after typhoid fever. In their course they 
differ in no way from other neuralgias. The only fact remark- 
able is that atrophy of the interossei and the " claw hand " 
may develop in their course even when there are no motor 
disturbances present. A relapse in a case of ulnar neuralgia 
may occur after an interval of years, but no satisfactory expla- 
nation for this has been discovered. 

Lastly, we have to consider in the upper arm the musculo- 
cutaneous and the circumflex nerves (Fig. 106), either of which 
may be affected by itself or in connection with other nerves of 
the plexus. The former supplies the coraco-brachialis, the 
brachialis anticus, and biceps ; the latter, the deltoid. 

Lesions of the motor fibres of the musculo-cutaneous, which 
are only met with independently after injury due to surgical 
operations, impair and completely prevent flexion of the fore- 
arm on the upper arm. In lesions of the circumflex, motion 
of the arm away from the trunk is difficult, and even rendered 
impossible, if, as often happens in the course of the disease, the 
deltoid atrophies. This atrophy is readily recognized by the 
flattening of the shoulder, and is often associated w T ith reaction 
of degeneration (cf. Windscheid, Neurol. Centralblatt, 1892, 7). 
Occasionally the participation of the sensory fibres of the cir- 
cumflex is more prominent ; the patients then complain of 
violent neuralgic pains (Heon, cf. lit.), which are aggravated if 
any attempt is made to move the arm. It is important in such 
cases to make a careful examination of the shoulder joint, and 
frequently we shall find a chronic inflammation here to be the 
cause of the neuritis. Recently, F. Schultze has carefully 
studied the so-called acroparesthesia (Deutsche Zeitschrift f. 
Nervenheilk., 1893, iii, p. 300). 

In other cases, again, we can not make out any organic 
changes in the joint, and we have to think of a joint neurosis. 
For information on this point the reader is referred to the 
chapter on Hysteria. A severe concussion, a fall upon the 
shoulder, which at first produces hardly any symptoms, may 
give rise to disease, lasting for years, in wmich both the joint 
and the nerves of the plexus take part. 
.23 



354 



DISEASES OF THE SPINAL NERVES. 



In any one of these affections of the nerves of the arm we 
should in the treatment, besides aiming at the removal of the 
cause if such be found, make use as soon as possible of the gal- 
vanic current. It is a mistake to lose time with other meas- 
ures, such as bathing, massage, rubbing, and the like. Where 
the electrodes are to be applied may be learned from the illus- 
trations, in which the motor points are accurately given. We 
need hardly say that, besides the electricity, various placebos, 
rubbing and passive motion, may be used to quiet the patient's 
mind. 

Not uncommonly several nerves of the brachial plexus are 
paralyzed at the same time. Duchenne was the first to de- 
scribe such instances in children in consequence of obstetrical 
operations, such as version and subsequent extraction, the 
Prague method of extraction, etc., and designated this form as 
" paralysie obstetricale infantile du membre superieur," or 
birth palsies. Independently of the French investigator, Erb 
has given us an excellent well-defined picture of such a paral- 
ysis. The lesion which affects the plexus gives rise to a simul- 
taneous paralysis of the deltoid, the biceps, the brachialis 
anticus, and the supinator longus, and the patient can neither 
move his upper arm away from the body, nor approach the 
forearm to the upper arm. The whole extremity hangs down 
flaccid, while the fingers and hand retain their mobility. The 
lesion in such cases must be situated at a point where the cir- 
cumflex and the musculo-cutaneous and the musculo-spiral are 
still close together — i. e., at about the exit of the sixth cervical 
nerve — between the scaleni, and it is from this so-called 
" Erb's " or "supraclavicular" point (cf. Fig. 107) that we are 
able to stimulate simultaneously all these four above-mentioned 
muscles. If the infraspinatus is also taken in, the arm is in 
a position of internal rotation, and can not be turned out- 
ward. 

This paralysis, which Erb has aptly termed " combined 
shoulder-arm palsy," is often a very tedious and troublesome 
affection. The longer it lasts the more the nutrition of the 
muscles suffers, and the most varied degrees of atrophy, which 
is often especially marked in the deltoid, are seen. On elec- 
trical examination we find that the faradic and galvanic excita- 
bility of the nerves, although not completely lost, is diminished, 
as is also the faradic excitability of the muscles, while the gal- 



LESIOXS OF THE BRACHIAL PLEXUS. 



355 



vanic excitability of the same has undergone qualitative as 
well as quantitative changes, a condition which Erb has desig- 
nated as partial reaction of degeneration. Sometimes, also, 
there is present complete reaction of degeneration (cf. page 
91). If the sympathetic is also implicated (Seeligmiiiler). the 
ensuing paralytic symptoms, contraction of the pupil, narrow- 
ing of the palpebral fissure, and retraction of the bulb on the 
affected side, are further sources of annoyance to the patient. 



X. dorsalis 

scapulas 

Circumflex 

nerve 

Post, thoracic 

nerve (M. ser- 

ratus magnus) 

Phrenic nerve 




Anterior thoracic 
nerve (J/~. pector.) 



Brachial plexus 



ErVs supraclavicular point 
Fig. 107. 



How the participation of the sympathetic is to be explained, 
whether, as Klumpke (cf. lit.) holds, by a lesion of the commu- 
nicating branch of the first dorsal, we can not decide. If the 
sensory fibres are also implicated, the patient complains, in 
addition to the motor, also of sensory disturbances, not only of 
great difficulty in moving the arm, but also of pains, numbness, 
and formication. 

The treatment, of course, consists in the use of electricity, 
galvanic stimulation from Erb's point, and the application of 



356 DISEASES OF THE SPINAL NERVES. 

the faradic brush, which, acting reflexly, often give very good 
results. 

Peculiar and very curious motor phenomena in the upper 
extremities are observed in connection with and as a direct 
consequence of certain callings. Such occur in cases where 
no particular exertion of the muscles might lead us to think of 
a peripheral lesion of the plexus as the result of overstrain, but 
in persons whose occupations bring into play complex, co-ordi- 
nated movements. Since in many cases — but by no means in 
all — a faulty co-ordination of the movements is the cause of the 
affection, we may for the present accept the name of " co-ordi- 
nation occupation neurosis," which was proposed by Benedikt, 
at the same time insisting upon the fact that it only fits a cer- 
tain small number of cases. 

Among the occupations which relatively frequently give 
rise to the disturbance in question the most important certainly 
is writing, and writer's cramp — mogigraphia, graphospasmus — 
is one of the nervous diseases to which most careful study has 
been devoted. Nevertheless, our knowledge is extremely lim- 
ited, and we must confess that we have not as yet got be- 
yond the description of the symptoms. The pathogenesis and 
therapeutics are terrce incognita. 

In the first place, we ought to state that only in a fractional 
number of cases have we to deal with a cramp or spasm ; more 
often the conditions are the following : The patient, after having 
for weeks, perhaps months, noticed that while writing the hand 
becomes tired more easily than before, finds one day that he is 
utterly unable to write another line without great strain ; as 
soon as the pen is taken into the hand the sensation of fatigue 
comes on ; hand and arm drop as if paralyzed, while at the 
same time the patient may complain of more or less intense 
pain in the forearm, upper arm, and possibly in the shoulder. 
The writer's cramp in such cases is in reality a writer's paral- 
ysis. In other instances, as soon as the penholder is clasped 
the hand begins to tremble and the handwriting becomes un- 
certain and tremulous, which is all the more striking because 
on examination the patient's hand, especially the right, proves 
to be quite steady if it is not used in writing. Sometimes there 
is an actual spasm when the penholder is seized, which attacks 
the muscles of the hand as well as those of the forearm, so that 
hand and arm make involuntary movements or they become 
stiff and immobile (clonic and tonic spasm). The pen is either 



LESIOA T S OF THE BRACHIAL PLEXUS. 



357 



irregularly jerked to and fro or firmly pressed against the 
paper ; in both cases writing is absolutely impossible. On 
further examination nothing else is discovered, and, what is 
more especially interesting, the patient is able to do anything 
else with his hands, even the finest work. He is able to draw 
(with a pencil), play the piano, etc. ; moreover, the electrical 
examination of the apparently seriously affected muscles sel- 
dom reveals anything abnormal worthy of mention. Dubois 
(Schweiz. Correspondenzbl., 1887, 5) found the excitability for 
both currents, especially in the thenar muscles, increased. 
Sensibility is, on the whole, normal. Pains only occur on 
forced attempts to write ; in short, the patient can do anything 
demanded of him except write. 

Analogous to the affections just described are the condi- 
tions of fatigue in the muscles of people, chiefly professionals, 
who play the piano a great deal. In them not only the right 
hand, but, especially in female patients, the left also is affected. 
Pain and weakness may become so marked in both hands that 
piano-playing has to be given up completely. This becomes 
the more necessary when the symptoms persist during rest as 
well, and not only when the patient is playing. Such disturb- 
ances are also noted in telegraph operators, cigar-makers, 
and in milkers of cows ; also, but rarely, in tailors it is pro- 
duced by the frequent handling of the heavy shears, etc. In 
all cases it is evident that the occupation is the sole cause, 
although we do not know how and upon what organs it acts 
injuriously. It is very unlikely that the disturbance is of a 
peripheral nature, the negative result of the examination of 
muscles and nerves and the uselessness of any treatment seem- 
ing to indicate this. We can not accept either the theory 
which attempts to explain the symptoms by a primary weak- 
ness of certain muscles and a secondary spasm of the antago- 
nists (Zuradelli), or that which assumes the spasm to be of 
a reflex nature, starting from the sensor}- nerves of the skin 
(Fritz) ; or, finally, the explanation that we have to deal with a 
disturbance in conduction of the nerve muscle apparatus used 
in writing ; but we are rather of opinion that the weakness and 
the motor disturbances of the upper extremity arising in con- 
sequence of the occupation are of a central nature and are to 
be referred to the brain cortex. The situation of the centres 
concerned in writing and in other movements which depend 
upon a co-ordinated action of the muscles of the hands is un- 



358 



DISEASES OF THE SPINAL NERVES. 



known. These centres in consequence of overexertion, but also 
often without any appreciable cause, are thrown into a state of 
paralysis or irritation which gives rise to corresponding dis- 
turbances in the extremities. Perhaps this may at times arise 
simply as the result of a general increased nervousness which 
may have a hereditary origin. It is evident that besides those 
affections which are due to a functional disturbance of the cor- 
tex there are those in which anatomical lesions, whether of the 
central organs or of the peripheral nerves, may be the cause of 
the same symptoms as those now under consideration. Thus 
we may sometimes meet with cases of old almost cured hemi- 
plegias in which as the only remaining disturbance a slight 
difficulty in writing or similar occupations may be present. 
The same may happen in slight disseminated scleroses of certain 
collections of fibres in the spinal cord, or, finally, as I have had 
occasion to observe repeatedly, in the initial stage of tabes, and 
the disturbance at the first glance may suggest to us writer's 
cramp. Hence we should, first of all, endeavor to decide 
whether the trouble is an independent affection or whether 
it is to be regarded merely as a symptom of an underlying 
disease. 

The prognosis is usually unfavorable. Only in the rarest 
instances are we able to afford the patients any decided last- 
ing relief, a fact of which we should inform the friends before 
taking charge of the case. Only when we are able to get hold 
of the patient in the earliest stages of the trouble and can in- 
sure him perfect rest and the removal of the exciting cause, 
such as writing, piano-playing, telegraphing, etc., for weeks and 
months, is it sometimes possible to effect an absolute cure. If 
this can not be done, and if the rest is not complete, the success 
of all our attempts becomes very uncertain and the result will 
usually be disappointing. We may try massage, as has been 
done also by some non-professional specialists with transient 
success. Galvanism, faradism, rubbing with different external 
applications, hydrotherapy, gymnastics, may be advised. The 
result is usually the same as if strychnine or atropine is in- 
jected hypodermically or if the different nervines be given inter- 
nally for months. Writing may be facilitated by using a pen- 
holder passed through a potato or through a wooden ball fitted 
to the hollow of the hand, or by using Nussbaum's bracelet. 
The advice to educate the left hand to write is always good 
because it gives the right hand a rest. Yet the value is by no 



LESIONS OF THE BRACHIAL PLEXUS. 359 

means lasting, because the motor disturbance, as a rule, shows 
itself soon in that hand also, a fact which is an additional argu- 
ment in favor of the central nature of the disease. 

The simultaneous affection'of several sensory nerves of the 
brachial plexus, analogous to the motor disturbance in the 
shoulder-arm palsy, is not common. When it does occur the 
pains are very violent and deprive the patient of the use of 
the extremity. The cervico-brachial neuralgia may affect all 
the sensory branches of the brachial plexus, so that the whole 
upper arm, forearm, and hand are painful ; but it may also be 
confined to the area of distribution of one nerve, often the 
musculo-spiral or median (cf. Nourric, De la nevralgie brachiale 
double, These de Paris, 1889). 

Painful points can sometimes be demonstrated in the region 
of the circumflex nerve over the scapula, of the median in the 
bend of the elbow, of the musculo-spiral in the lower third of 
the humerus, and of the ulnar at the internal condyle. Vaso- 
motor and trophic changes may be entirely absent, yet the 
skin of the fingers not rarely looks glossy and atrophic (" glossy 
fingers"). Here, again, traumatism, mechanical pressure — by 
tumors, aneurisms, etc. — are the most prevalent causes of the 
neuralgia. It may occur reflexly after amputation of the 
ringers or the forearm. A bilateral neuralgia of this kind is 
suggestive of a spinal disease, more especially of pachymenin- 
gitis cervicalis hypertrophica. 

The treatment is in the main the same as in other neural- 
gias. Besides narcotics the electrical treatment should be be- 
gun as soon as possible. Descending currents through the 
diseased nerve, as well as the application of the anode over the 
affected plexus, are to be recommended. The faradic brush is 
usually borne well and is of use, although the manipulation 
itself may not be very agreeable to the patient. In rare in- 
stances we must have recourse to energetic counter-irritants 
to the skin. We have repeatedly made very successful use of 
the points de feu with Paquelin's cautery. 

Parsesthesias and anaesthesias are quite common in the dis- 
tribution of the brachial plexus. They are not always con- 
fined to one nerve. Upper arm and forearm, the hands also, 
are frequently affected, particularly when the occupation ne- 
cessitates overexertion of them — e. g., in brick-makers. Again 
they are caused by the action of cold and hot water, often 



360 DISEASES OF THE SPINAL NERVES. 

also by water containing- lye (anaesthesia lavatricum, and the vial 
des bassins of the women engaged in unwinding the silk from 
the cocoons in the silk-spinning mills, etc.). For such patients 
the only remedy lies in abstention from this kind of work. 

LITERATURE. 
Lesions of the Cervical Plexus. 

Peter, M. Neuralgia phrenica. Arch, gener., 1871, 6me ser., xvii, p. 303. 
Erb. Handbuch der Krankheiten des Nervensystems. 2. Aufl., 1876, ii, pp. 

124, 125. 
Striimpell. Loc. cit., 1887, p. 32. 
Eichhorst. Loc. cit., 1887, pp. 47, 79, 82. 
Stevenson. Case of Persistent Hiccough ; Necropsy; Remarks. Lancet, 1883, 

i, 1043. 

Lesions of the Brachial Plexus. 

1. Paralysis of the Serratus. 

Berger, O. Die Lahmung des Thoracicus longus. Habilitationsschrift, Bres- 

lau, 1873. 
Brack. Ein Fall von Serratuslahmung nach acuter Krankheit (Typhoid). In- 

aug.-Dissert., Vratisl., 1873. 
Lewinski. Ueber die Lahmung des Serratus amicus major. Virchow's Archiv, 

1878, Ixxiv, 4, p. 473. 
Lewinski. Zur Diagnose der Serratuslahmung. Virchow's Archiv, 18S1, lxxxiv, 

1, p. 71. 
Baumler. Isolirte Lahmung der Serrat. ant. maj. Arch. f. Psych, und Nerven- 

krankheiten, 1882, xiv, 3, p. 722. 
Dixon Mann. Serratus Paralysis. Lancet, February, 1884, i, 5, 6. 
Hoffmann. Isolirte periphere Lahmung des Nerv. suprascapul. sinistr. Neurol. 

Centralbl., 1888, 9. (Pains and Atrophy of the Muscles supplied by the 

Nerve.) 
Bunting, Ross R. Paralysis of the Serratus Magnus. Journal of Nerv. and 

Merit. Diseases, 1890, xv, p. 67. 

2. Musculo-spiral Paralysis (not including the Saturnine Form). 

Fischer. Zur Lehre von der Lahmung des N. radialis. Deutsches Arch. f. klin. 

Med., 1876, xvii, 4, 5, 392. 
Onimus. Gaz. hebdom., 1871, 2me ser., xv, 25. 
Whitson. Musculo-spiral Paralysis in Consequence of Pressure exerted by a 

Piece of Bone. Edinb. Med. Journ., 1882, xxvii, p. 724. 
Boyer. De la paralysis du nerf radial par compression temporaire. These de 

' Paris, 1883. 
Joffroy. Du role de la compression dans la production de la paralysie radiale. 

Compt. rend, gener., May 14, 1884, p. 284. 
Arnozan. Gaz. hebd., 1885, xxxii, 2, 3. — E. Remak. Berliner klin. Wochenschr., 

1885, xxii, 5. — H. Neumann. Neurol. Centralbl., 1885, iv, 4. — Falkenheim. 

Mittheilungen aus der med. Klinik zu Kcnigsberg, 1888. (Musculo-spiral 

Paralysis after Subcutaneous Injection of Ether.) 



LESIOiVS OF THE BRACHIAL PLEXUS. 



361 



Vulpian et Dejerine. Recherches cliniques et experimentales sur la paralysie 
radiale. Compt. rend. hebd. de la Soc. de Biol, 1886, 15, p. 187. 

Scheiber, H. Ein Fall von schwerer complicirter Schlaflahmung am linken 
Arme. Neurol. Centralbl., 1886, v, 15. 

Kbbner, H. Ein P'all von gleichzeitiger traumatischer (Druck) Lahmung der 
Nervi radial., uln. und median, sinistr. Deutsche med. Wochenschr., 1888, 
10. 

Gluck. Sitzung der Berliner Gesellschaft f. Psych, u. Neivenkrankheiten 
vom 9 Juli, 1888. (Traumatic Musculo-spiral Paralysis cured by Sec- 
ondary Nerve Suture.) 

3. Median and Ulnar Paralysis. 
Bernhardt. Ueber den Bereich der Sensibilitats-Storung an Hand und Finger 

bei Lahmung des Medianus, sowie zur Pathologie der Radialisparalysen. 

Arch. f. Psych, und Nervenkr., 1875, v, 2. 
Tilden. Trophoneuroses after Injury to the Median Nerve. New York Med. 

Record, September 11, 1886, xxx, p. 30, 4. 
McNaught. Ulnar Neuralgia. Brit. Med. Journ., April 30, 1887, p. 933. 
Poore. Lancet, September, 1882, ii, io, 12. 
Leudet (de Rouen). Gaz. med. de Paris, September 15, 1883. 
Hess, Julius. Ueber Temperaturen und deren Messung bei Ulnarislahmungen. 

Berliner klin. Wochenschr., 1886, xxiii, 30. 
Ballet, G. Accidents consecutifs a la compression habituelle du cubital chez un 

ouvrier employe a ouvrager le verre. Revue de med., 6, 1885. 
Philiotis. De la nevrite peripherique du cubital consecutive a la rievre typhoide. 

These de Paris, 1885, No. 119. 
Eulenburg. Ueber Lahmung durch polizeiliche Fesselung (Arrestantenlahm- 

ung) der Hand. Neurol. Centralbl., 1889, 4. 
Rieder. Medianus- Neuritis. Munch, med. Wochenschr., 12, 1889. 
Stenzel. Deutsche med. Wochenschr., i8i8, xiv, 31. (Four Cases of Trauma- 
tism of the Ulnar Nerve.) 

4. Musculo-Cutaneous and Circumflex Paralysis. 

Heon. De la nevralgie circonflexe ou axillaire. These de Paris, 1882, No. 277. 
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1885, No. 371. 
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Wochenschr., 1888, 21, 22. 
Bruns. Isolirte Lahmung der linken Flex. poll. long, durch Ueberanstrengung 

(Drummer's Paralysis). Neurol. Centralbl., 1890, 12. 
von Zander. Trommlerlahmung. Inaug.-Dissert, Berlin, 1891. 

5. Plexus Paralysis — Combined Shoulder-arm Palsy. 

Bernhardt. Beitrag zur Lehre von den Lahmungen im Bereiche des Plexus 

brachialis. Zeitschr. fur klin. Med., 1882, iv, 3, p. 415. 
Vierordt. Zwei Falle von der Form der " combinirten Schulterarmlahmung." 

Neurol. Centralbl., 1882, 13. 
Klumpke. Contribution a 1 etude des paralysies radiculaires du plexus brachial. 

Revue de med., Juillet-Sept., 1885. 



362 DISEASES OF THE SPINAL NERVES. 

Vinay. Paralysies radiculaires superieures du plexus brachial, d'origine profes- 
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Bernhardt. Neurol. Centralbl., 1886, 6, p. 141. 

Rose. Deutsche Zeitschr. f. Chirurg., 1886, xxiv, 3, 4. 

Muralt. Einige, zum Theil chirurgische Lahmungen im Bereiche des Plex. 
brachialis. Schweiz. Correspondenzbl., 1888, xviii, 15. 

Middeldorpf. Wiener med. Wochenschr., 1888, 14. (Pressure Palsy of the 
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Jolly. On Birth Palsies. British Med. Journ., 1889, 6. 

Arens. Beitrag zur Pathologie der Entbindungslahmungen. Inaug.-Dissert., 
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Leszynsky, W. A Contribution to the Clinical Study of Spontaneous Degen- 
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Schaefer (Ovvinsk). Ueber Arbeitsparesen. From Mendel's Polyclinic. Inaug.- 
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(Klumpke's Paralysis). Deutsche Zeitschr. f. Nervenhk., 1891, i, 5, 6. 

Onanoff. Cas de paralysie radiculaire, brachiale totale. Arch, de Neur., 
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d'Astros, Leon. L'avenir des paralysies obstetricales du membre superieur. 
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Rieder. Die Steintragerlahmung. Munch, med. Wochenschr., 1893, 7. 

Braun, H. Die Drucklahmungen im Gebiete des Plexus brach. Deutsche med. 
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6. Co-ordination Occupation Neuroses. 

Napias. Photographer's Cramp. Revue d'Hygiene, November, 1879. 
Mbbius. Berliner klin. Wochenschr., 1880, xvii, 21. (Cramp from Playing the 

Zither.) 
Dally. Journ. de Therapeut., 1882, 3, 4. 
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1882. 
Poore. Brain, 1883, p. 233. (Sawyer's Cramp.) 
Vigouroux. Progr. med., 1882, x, 3. 
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Bracelet.) 
Villemin. Arch. d. med. et de pharm. milit., 1883, pp. 91-95. 
Poore. A Case of Hammerman's Cramp. Lancet, August 21, 1886, 8. 
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1887. 
Poore. The Lancet, 1887, 3322. (Writer's Cramp.) 
Gaborian. Contribution a l'etude des spasmes professionnels. Th£se de Paris, 

1887. 



DISEASES OF THE DORSAL NERVES. 



363 



Poore. On Certain Conditions of the Hand and Arm which interfere with the 

Performance of Professional Acts, especially Piano-playing. British Med. 

Journ., February 26, 1887. 
Zenner. Berliner klin. Wochenschr., 1887, 17. 
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Physiol, norm, et Pathol., 1883, 7. 
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inaugur.. Paris, 1884. 
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Squire, J. Edward. Some Cases of Local Numbness of the Extremities, with a 

Comparison between Local Syncope and Night Palsy. Lancet, December, 

1885, ii, 23. 
Bernhardt. Ueber eine weniger bekannte Neurose der Extremitaten besonders 

der oberen. Centralbl. f. Nervenhk., 1886, ix, 2. 
Remak. Zur Pathologie des Melkerkrampfes. Deutsche med. Wochenschr., 

1889, 13, p. 158. 
Weiss, M. (Wien). Die Elektrotherapie der peripheren graphischen Stbrungen. 

Centralbl. f. d. ges. Therapie, 1891, ix, 4, p. 193. 

II Diseases of the Dorsal Nerves. 

The anterior (ventral) divisions of the twelve dorsal nerves are 
called the intercostal nerves, since they run in the intercostal spaces. 
They supply the intercostal muscles, the levatores costarum, the 
serrati postici, and the three broad abdominal muscles. To the in- 
tegument of the chest and abdomen they supply cutaneous branches. 
The posterior divisions of the dorsal nerves are divided into internal 
and external branches. The former are distributed to the deep 
muscles of the back, sending nerves to the rhomboidei and the latis- 
simus dorsi ; the latter, passing between the longissimus dorsi and 
the sacrolumbalis, also furnish numerous muscular branches, and, to- 
gether with the internal, supply the skin of the back as far down as 
the crest of the ilium. 

The sensory as well as the motor fibres of the dorsal nerves 
may become the seat of disease, but, and this is practically of 
much importance, the anterior, the intercostal nerves, are more 
subject to sensory disturbances, while the diseases of the pos- 
terior branches are almost exclusively motor affections. 

The disease of the anterior branches, the so-called intercos- 
tal neuralgia, is found with relative frequency in the female 
sex, especially in those of middle age. ^Etiologically, oc- 
cupation and hard work in general are of some importance. 
Servant girls and women of the poorer classes suffer more 



364 DISEASES OF THE SPINAL NERVES. 

frequently than others. I have seen many such instances, and 
have found it besides in the course of phthisis pulmonalis when 
associated with peripheral neuritis. Traumatism, aortic aneu- 
rism, and spinal affections, may also give rise to intercostal 
neuralgia. 

The pain appears in paroxysms and attacks more frequently 
the left than the right side, and almost exclusively the anterior 
or lateral, rarely the posterior, portion of the nerve trunks. It 
often follows the course of the nerve and at times reaches a 
degree of intensity most distressing to the patient. The re- 
spiratory movements, more especially coughing and sneezing, 
cause great agony. Three tender points can usually be demon- 
strated — one close to the vertebral column, one in the middle 
of the course of the nerve, and one close to the sternum — 
called respectively the vertebral, lateral, and sternal points. 
The fact that frequently after cessation of the pain a herpes 
zoster appears is of great interest, although the question 
whether we have to regard the latter as a genuine trophic dis- 
turbance or simply as an extension of the inflammation from 
the nerve endings to the skin, as Gubler thinks, is still unset- 
tled. For the prognosis it is without significance. In all cases 
of intercostal neuralgia the prospect for complete recovery is 
slight. Although we may succeed sometimes in cutting short 
the individual attacks, we can never be certain that they will 
not recur, and there are persons who all their life long are 
condemned to suffer from this disease. 

The diagnosis is not always simple. Rheumatism of the 
chest muscles can easily be taken for intercostal neuralgia, and 
vice versa. In such cases we shall find it useful to observe 
whether motion has any influence on the pain or whether this 
exists independently. If there is a history of traumatism, neu- 
ritis is always to be thought of, only we must beware of being 
deceived by malingerers, and to avoid this the condition of the 
abdominal reflex and the pupil should be examined into. The 
former in the case of neuritis is increased, the latter often di- 
lated on the side of the pain. This fact was first established 
by Seeligmuller, and shows that the sympathetic is often im- 
plicated here just as in the affections of the brachial plexus 
(Deutsch. med. Wochenschr., 1887, 45). 

In the treatment morphine plays the most important role, 
and, as a matter of fact, it is of much more value than the much- 
lauded subcutaneous injections of osmiumine (one syringeful of 



DISEASES OF THE DORSAL NERVES. 



365 



a one-per-cent solution at a dose), for this not only frequently 
disappoints us, but also produces local troubles, small abscesses, 
etc., so that the patient is left almost in a worse condition than 
before. The faradic brush, the "points de feu" with Paquelin's 
cautery, blisters applied to the painful points, may be tried ; 
but, on the whole, these means effect but little, 

Among the intercostal neuralgias, the so-called mastodynia 
(the irritable breast of Cooper), a neuralgia of the mamma, is 
to be included. This is a not very frequent affection of the 
female after puberty, and may be connected with lactation. It 
is a very painful and distressing trouble, against which usually 
all remedies are tried in vain, so that in desperate cases the 
patient herself suggests amputation of the breast to get rid of 
the dreadful suffering. The aetiology is obscure. Traumatism 
is rarely the cause. Ill-fitting corsets may have some influence, 
but women with well-developed and those with small breasts 
are equally liable to the affection. The hyperassthesia of the 
skin often hinders a careful examination by palpation. With 
the tips of the fingers we should endeavor to determine 
whether there are hard nodules in the tissue, which to the 
inexperienced often suggest beginning carcinoma. In some 
cases my patients have derived some transient benefit from 
suspension of the breast and the application of hot cloths. 
Here also morphine is indispensable (cf. Terrillon, Des neu- 
ralgies du sein ; Progr. med., 1886, xiv, 10). 

The motor disturbances affecting the muscles of the back 
supplied by the posterior branches of the dorsal nerves are 
generally paralyses. We are far from being familiar with the 
symptoms of the affections of every one of these muscles, and 
must content ourselves for the present with mentioning the 
paralysis of the erector spinas, the sacro-lumbalis, and the lon- 
gissimus dorsi, which may be affected in the lumbar, dorsal, 
or cervical portion of the vertebral column. Bilateral paraly- 
sis causes curvature of the spine backward (kyphosis), unilat- 
eral paralysis lateral curvature (scoliosis). Paralysis or pare- 
sis of the erectors in the lumbar region gives rise to a charac- 
teristic walk and a characteristic position of the body. The 
upper part of the body is bent strongly backward, so that the 
lumbar part of the vertebral column is markedly curved for- 
ward. If by any movement the upper part of the body is 
brought forward so that its centre of gravity is no longer be- 



3 66 



DISEASES OF THE SPINAL NERVES. 



hind that of the whole body, the patient falls forward, or, if the 
patient sits on the floor, he has the greatest difficulty in getting 
up. The manner in which he raises himself is so characteristic 
of paralysis of the erector muscles that we have represented it 
in Figs. 108 to in. The patient first gets upon all fours, and 
then climbs, as it were, with his hands up his own legs, con. 





Fig. 108. 



Fig. 109. 





Fig. no. Fig. in. 

Figs. 108-111 illustrate the manner in which a child whose erect ores spinae are paralyzed gets 
up from the ground. (After Gowers.) 

stantly endeavoring to bring the upper part of the body as far 
back as possible by movements in the shoulders and the arms 
so that the abdominal muscles may resume the duty of balanc- 
ing the body. This mode of getting up can best be studied in 
pseudo-hypertrophic paralysis. 

III. Diseases of the Lumbar Nerves. 

The posterior lumbar nerves are, like the dorsal, divided into 
outer and inner branches, which are distributed to some of the mus- 
cles of the back and the skin of the lumbar and gluteal region. The 
anterior, by far the stouter, are connected each with the correspond- 
ing ganglion lumbale of the sympathetic. They form the lumbar 
plexus which lies behind and in the psoas muscle. Its branches are 
(Fig. 112): (1) The ilio-hypogastric nerve, for the transversalis and 
the internal oblique; (2) the ilio-inguinal, for the skin of the pubes 
and the genitals (N. scrotales et labiales anteriores) ; (3) the genito- 
crural, which divides into the external spermatic or genital branch 



LESIOXS OF THE LUMBAR NERVES. 



367 



and the lumbo-inguinal or crural branch, the former supplying the 
spermatic cord, the cremaster muscle, and the testis, the latter the 




Fig. 112.— Diagrammatic Outline of the Lumbar and Sacral Plexuses. DXII, 
last dorsal nerve. LI-V, the five lumbar nerves. S I-V, the five sacral nerves. CI, 
the coccygeal nerve. 1, ilio-hypogastric nerve. 1', ilio-inguinal nerve. 2, genito-cru- 
ral nerve. 2', external cutaneous nerve of the thigh, crur, anterior crural nerve, obt, 
obturator nerve. 3, superior gluteal nerve, sc, great sciatic nerve. 4, small sciatic 
nerve. 4', inferior gluteal nerve. 5, inferior pudendal nerve. 5', posterior cutaneous 
nerve of thigh and leg. 6, 6, branch to obturator internus and gemellus superior. 6', 6', 
branch to the gemellus inferior, quadratus femoris, and hip joint. 7, twigs to the piri- 
formis. 8, pudic nerve. 9, visceral branches. 9', twig to the levator ani. 10, perforat- 
ing cutaneous nerve, it, coccygeal branches. 



3 68 



DISEASES OF THE SPINAL NERVES. 



skin in the inguinal region ; (4) the external cutaneous, for the skin 
down to the knee; (5) the obturator, which gives off a posterior 
branch to the obturator ext. and adductor magnus and an anterior 
branch to the skin of the inner side of the thigh ; and (6) the anterior 



, \ l se \ 

r f\ A m 



\ 




pep 



\irpm,: 



<J> 



KftT: 



->^0> 



SO/ 



Fig. 113. Fig-. 114. 

Areas of Distribution of the Cutaneous Nerves of the Lower Extremity. 
(After Henle.) Fig. 113, anterior surface. 1, middle cutaneous nerve. 2, external 
cutaneous nerve. 3, ilio- inguinal nerve. 4, genito-crural nerve. 5, external spermatic. 
6, posterior cutaneous nerve. 7, obturator nerve. 8, internal saphenous nerve. 9, com- 
municating peroneal. 10, superficial peroneal, n, deep peroneal. 12, communicating 
tibial. Fig. 114, posterior surface. 1, posterior cutaneous nerve. 2, external cutaneous 
nerve. 3, obturator nerve. 4, median posterior femoral cutaneous. 5, communicating 
peroneal. 6, saphenous nerve. 7, communicating tibial. 8, plantar cutaneous. 9, me- 
dian plantar nerve. 10, lateral plantar nerve. 



crural (five millimetres in width), giving muscular branches to the 
anterior periphery of the thigh and having also cutaneous branches 
— middle cutaneous, internal cutaneous, and the long or internal 
saphenous nerve (cf. Figs. 113 and 114). 



LESIOXS OF THE LUMBAR NERVES. 



369 



All the nerves of this plexus contain sensory as well as 
motor fibres, and hence may be affected in both ways. However, 
these affections do not often appear independently, whereas 
they are frequently observed as symptoms of central, more espe- 
cially of spinal, diseases, and, above all, of tabes. Our descrip- 
tion of them, therefore, will here be very brief. 

Among the sensory disturbances we have first to mention 
the lumbo-abdominal neuralgia, in which the hip joint is affected 
in much the same way as the shoulder joint in cervico-brachial 
neuralgia, so that the whole lumbar region down to the but- 
tock is intensely painful. Of greater practical importance is 
what Cooper has described as " irritable testicle," neuralgia 
spermatica or neuralgia of the testicle, which either only forms 
a part, of the lumbo-abdominal neuralgia, or, as Eulenberg and 
others assume, is a neuralgia of the sympathetic nerve. The 
spontaneous pain and the tenderness may attain such a degree 
as to lead to temporary psychical disturbances. Generally 
only one testicle is affected, and most of the instances are 
found in young people. Benda has cured a case of this neural- 
gia by the application of a bandage which exercised a continuous 
pressure upon the inguinal region ; it is impossible to explain 
the modus operandi of this measure (Berlin, klin. YVochen- 
schr., 1890, 38). Further, we would mention the crural neural- 
gia, and the obturator neuralgia, affections which manifest 
themselves by pain following exactly the course of the respect- 
ive nerves. The existence of tender points is not constant and 
their seat varies. 

The treatment must be carried out according to the prin- 
ciples which we shall describe later in our account of sciatica. 

Even less frequently than the sensory do the motor disturb- 
ances occur by themselves. If present, they are mostly of 
spinal, rarely of peripheral, origin. Paralyses in the distribu- 
tion of the crural nerve, which interfere with the function of 
the ilio-psoas and the quadriceps, make it impossible for the 
patient to bend the thigh at the hip joint and to extend the leg 
after it has been flexed on the thigh. Paralyses of the obtura- 
tor nerve interfere with the adduction of the thigh and the 
patient is no longer able to cross the affected leg over the 
other. On the other hand, a contracture of these muscles fol- 
lowing myelitis may necessitate the resection of the nerve, an 
operation which may be followed by immediate relief (Lauen- 

stein, Centralbl. f. Chir., 1892, 11). Fere and Perruchet have 
24 



37o 



DISEASES OF THE SPINAL NERVES. 



published an exhaustive study upon the traumatic origin of 
neuralgia of the obturator nerve (Revue de Chir., 1889, ix, 7, 
p. 574). Disorders in the nerves of the gluteus, the tensor vagi- 
nas femoris, and the pyriformis impair rotation of the thigh 
inward and outward. Abduction is also hindered, while the 
actions of walking, standing, and more especially climbing 
stairs, are performed awkwardly. For details the reader is 
referred to Duchenne-Wernicke, pages 261 and following, where 
the normal and pathological physiology of these muscles is 
carefully discussed. 

IV. Diseases of the Sacral and Coccygeal Nerves. 

The posterior small branches of the sacral nerves, four of which 
leave the vertebral canal through the posterior sacral foramina and 
the fifth through the foramen between the sacrum and coccyx, form 
numerous anastomoses, and thus constitute what is known as the 
posterior sacral plexus. The anterior, much larger, branches pass 
into the pelvis, where the first three and a part of the fourth, to- 
gether with the lumbo-sacral cord (resulting from the junction of the 
fifth and a part of the fourth lumbar nerves), go to form the (ante- 
rior) sacral plexus. The plexus is triangular in form and rests upon 
the pyriformis muscle. The several nerves unite without much in- 
terlacement into an upper, large, and a lower, small, cord or band. 
The upper is formed by the union of the lumbo-sacral cord with the 
first and second and the greater part of the third sacral nerves and 
is continued into the great sciatic nerve. The lower becomes the 
pudic nerve. The plexus gives origin to a number of collateral 
branches — the superior and inferior gluteal, the small sciatic, and 
perforating cutaneous nerves and branches to the pyriformis, obtura- 
tor internus, gemelli, and quadratus femoris. The great sciatic nerve, 
the largest nerve of the body, divides into the internal popliteal and 
external popliteal or peroneal, the latter again dividing into the 
anterior tibial and musculo-cutaneous, the former, which becomes 
the posterior tibial, terminating in the internal and external plantar 
nerves. The pudic nerve divides into the inferior hemorrhoidal, the 
perineal nerve, and the dorsal nerve of the penis or clitoris. 

The anterior branch of the coccygeal nerve is distributed to the 
integument over the back part and the side of the coccyx. It is 
joined by a branch from the fifth sacral nerve, while the posterior 
division is lost in the fibrous structures on the back of the coccyx. 

The affections of the sacral plexus, which appear independ- 
ently of any other disease, are chiefly sensory in nature. Mo- 
tor disturbances, although they are perhaps numerically as 



LESIONS OF THE SACRAL PLEXUS. 



371 



common as the former, are in the great majority of instances 
symptomatic of spinal disease. Careful studies upon the 
lesions of the sacral and lumbar plexus have been published by 
Charles K. Mills, in the Medical News, June 15, 1891. 

Sciatica. 

Among the sensory disturbances there is especially one 
disease which, owing to its relative frequency and obstinate 
resistance to treatment, has attained to much practical impor- 
tance — namely, the affection of the sensory fibres of the sciatic 
nerve, the sciatic neuralgia or sciatica, malum Cotunnii (Co- 
tugno, 1764). This may, as autopsies have shown, be due to 
an organic disease of the nerve, a genuine neuritis, or to a func- 
tional neurosis. In the former there exist varicose dilatations 
of the blood-vessels of the nerve, swelling, increase in volume, 
alterations in consistency, and a collection of serous exudation 
in the nerve sheath (Cotugno, Jasset). In the latter no anatom- 
ical changes can be detected. The neuritis may be due to dis- 
ease of the neighboring structures, to a tenosynovitis in the 
lower leg (Erb), to affections of the vertebras (spondylitis, car- 
cinoma), or may appear independently, in which case, leaving 
cold out of consideration for a moment, we have usually to 
deal with mechanical injuries, either as the consequence of 
wounds, fractures, or as the result of protracted pressure (tu- 
mors of the pelvis, aneurisms, hernia, uterus gravidus, engorge- 
ment of the venous plexus of the pelvis, habitual constipation, 
etc.). The occupation must, moreover, be taken into consid- 
eration in the aetiology of sciatica. It may exert an injurious 
influence in one of two ways, either through the overexertion 
which it entails or through the exposure to frequent sudden 
changes of temperature. Of the former we have instances in 
those who work with the sewing machine for weeks and 
months for several hours a day, and in those who are always 
lifting heavy weights (stevedores, blacksmiths, etc.). To this 
class is thought to belong " le lumbago des forgerons" de- 
scribed by Maisonneuve (Hirt, Krankheiten der Arbeiter, iv, 
90). Of the latter we have instances in puddlers and those 
who work at smelting furnaces, etc. Sciatica is frequently 
seen among such people, and seems to affect more commonly 
the left leg, probably because in throwing the coal into the 
furnace it has to be extended more forcibly (Chiene, of Edin- 
burgh, and Hirt). 



372 



DISEASES OF THE SPINAL NERVES. 



As a symptom sciatica is often seen in spinal affections 
(myelitis, spinal meningitis), in diseases of the general nervous 
system, especially in tabes, where it often appears bilaterally, 
also in diabetes. As a sequela it has been described as follow- 
ing typhoid fever. Whether malarial intoxication can ever be 
the cause of it is uncertain. It is sometimes seen in the course 
of syphilis. In lead and mercury poisoning it plays an entirely 
secondary role. 

Symptoms. — -Among the symptoms of sciatica pain is the 
most important. The motor disturbances which sometimes 
occur in the course of the disease — tremor, clonic spasmodic 
movements, the difficulty and awkwardness in moving which 
interfere to a greater or less extent with standing and walking 
— have to be looked upon simply as the result of the pain. 
This varies greatly. At first it may be dull and quite bearable, 
but later boring in character, extending over the whole lower 
extremity and persisting without intermission, so that it con- 
stantly occupies the attention of the patient and forces him to 
a frequent change of position ; or, again, it may appear in at- 
tacks, with intervals of comparative ease, so that the patient 
feels fairly comfortable and is able to follow his occupation. 
During the seizures it may be of such excruciating intensity 
that it can only be compared with Fothergill's faceache or the 
lancinating pains of tabes. 

The patient suffers usually more intensely at night after 
going to bed, or at least he complains more at that time, often 
because he can not bear the extension of the leg, often perhaps 
because his attention is then less liable to be distracted. Yet 
even in the daytime the pain may reach a considerable pitch, 
especially when the patient has been making attempts to walk 
or has been standing too much. He may have perfect ease for 
hours when lying quietly, and yet a few moments of flexion 
and extension of the affected extremitv are sufficient to throw 
him back into the most distressing condition. The extent of the 
pain also varies ; generally it is felt over the whole posterior 
surface of the thigh and the distribution of the external poplit- 
eal nerve. It may radiate into the region of the healthy sciatic 
and the lumbar plexus of the affected side. The posterior 
tibial nerve usually remains intact. Examination almost always 
discloses the existence of tender points, one, for instance, at 
the exit of the nerve from the pelvis, one at the lower margin 
of the gluteus, one in the popliteal space, one on the capitulum 



LESIONS OF THE SACRAL PLEXUS, 



373 



fibulas. There may be others, but their occurrence is uncer- 
tain and their position changeable. 

Sometimes patients with sciatica are seen to put all their 
weight upon the healthy leg in order to diminish the pain in 
the affected extremity. This causes the trunk to be bent to- 
ward the healthy side and the costal margin to approach the 
ilium, a position which may become so habitual that a genuine 
scoliosis may be developed, the convexity of which is directed 
toward the healthy side ; in exceptional cases the reverse is 
the case — namely, the convexity of the curvature is directed 
toward the affected side. Remak (cf. lit.) is of opinion that the 
patient is able to convert the "normal" into the "abnormal" 
scoliosis as his comfort may demand, while Brissaud regards 
the abnormal position as the result of a reflex spasm. I have 
known several cases in which this secondary scoliosis per- 
sisted after considerable improvement of the primary affection, 
whereas in other instances I have seen it disappear when the 
cure of the sciatica was complete. 

If we have to deal with a genuine neuritis trophic changes 
will be found to develop, especially more or less marked atro- 
phy of the muscles in various regions supplied by the sciatic 
nerve (Guinon et Parmentier and others), with reaction of de- 
generation (Xonne). The patellar reflex seems in such cases 
to be considerably diminished. An exaggerated knee jerk in 
the course of a peripheral neuritis has, on the whole, to be 
looked upon as exceptional (Strumpell, Mobius). In sciatica I 
have never seen it. If the trouble is purely functional the 
muscles and reflexes remain, even after years of suffering, 
unaltered. Other sensorv changes — diminution of the sensi- 
bility, anaesthesias, paresthesias — occur, but take a very sec- 
ondary position to the dominating feature of the disease, which 
is pain. 

Course. — The course as well as the duration varies greatly, 
but we may state as an undeniable fact that it is exceptional 
to find cases which last but a short time and end with com- 
plete recovery. Mostly it is a question of months and years 
before any decided lasting improvement is brought about. 
On the other hand, remissions are not rare. They may last 
for months, and the condition of the patient may be such that 
he begins to be confident of a permanent cure, when suddenly, 
often without any appreciable cause, sometimes in consequence 
of a long walk, the pain again makes its appearance with un- 



374 



DISEASES OF THE SPINAL NERVES. 



diminished intensity and the treatment has to be started all 
over again. The more frequent such relapses, the more gloomy 
becomes the outlook for complete recovery. 

Diagnosis. — Great care should be exercised in the diagno- 
sis, and we should first endeavor to decide whether the trouble 
has to be regarded as an idiopathic affection or as a symptom 
of another malady, and more especially in bilateral sciatica 
should we be on the lookout for a spinal disease or a disease of 
the general nervous system, such as tabes. The examination 
of the urine for sugar should never be omitted. If this proves 
negative, and if we can exclude general nervous diseases with 
certainty, we should proceed to analyze the pain, to examine 
into its nature, the time of its occurrence, its seat and extent, 
and should keep in mind that there are other than nervous af- 
fections that are associated with violent pain in the lower ex- 
tremities; for instance, acute rheumatism of the lumbar muscles, 
lumbago, also inflammations in the hip joint, chronic hip dis- 
ease, malum coxse senile, as well as gouty affections and psoas 
abscesses. In all such instances the immobility of the extrem- 
ity, which also exists in a pure sciatica, makes the examination 
difficult, and only after persistent repetitions shall we be able 
to obtain a clear idea as to the true nature of the trouble. Al- 
though it may be going too far to say with Hutchinson that 
out of twenty cases diagnosticated as sciatica in nineteen there 
exists no trouble whatever in the nerve (Medical Times and 
Gazette, 1882, vol. i, No. 1648, page 35), there can be no ques- 
tion but that here many diagnostic sins are committed and 
that there are many cases called sciatica after a superficial ex- 
ploration which later prove to be something entirely different. 

Treatment. — The treatment of sciatica should vary accord- 
ing as the neuralgic pains constitute merely a symptom or 
result from an independent affection of the nerve itself. In the 
former case our therapeutic measures, of course, must be di- 
rected against the underlying disease (diabetes, tabes, syphilis, 
etc.). If we have to deal with sciatica as an affection by itself 
our treatment should be systematic and carried out on defi- 
nite lines. Our first rule should be never, or at any rate only 
in exceptional instances, to withdraw blood. If there are old 
scybalous masses in the bowel which press upon the nerve 
and thus cause the pain, considerable and lasting improvement 
may be brought about by the removal of these, and a course 
at Carlsbad or Marienbad may cure sciatica in such cases 



LESIONS OF THE SACRAL PLEXUS. 



375 



more quickly and surely than the most careful use of electrici- 
ty. Next, especially when we have grounds for suspecting 
an inflammatory condition of the nerve, we should try the ap- 
plication of counter-irritants to the skin, fly-blisters or the 
so-called points de feu (with Paquelin's thermo-cautery). The 
former more particularly, which have been used by Cotugno 
and Valleix, deserve to be recommended, as they prove gen- 
erally very effectual if used early in the disease ; they may be 
applied along the course of the nerve on the thigh or in the 
sacral region (Anstie). Less benefit is usually derived from 
irritating inunctions and plasters, which may, however, be 
given a trial ; for instance, we may employ one of Betz's plas- 
ters — empl. oxycroc. 15.0 (§ss.) ; arg. nitr. pulv., 1.0 (grs. xv) — 
allowing it to remain on the skin until it drops off of its own 
accord. Among other drugs for inunctions besides veratrine 
[0.1 : 10 lard] the narcotics (preparations of opium, belladonna, 
hyoscyamus) may be useful. Finally, the chloride-of-methyl 
spray may be recommended. This, however, should be used 
with great care ; otherwise it may be followed by a cellulitis, 
erysipelas, or even gangrene. The desired effect does not al- 
ways follow (cf. Steiner, Deutsche Med.-Ztg., 1891, 102, p. 

1 1 5 8). 

From internal medicines I have never seen an) 7 lasting good 
results; besides antipyrin and antifebrine, iodide of potassium 
has been used from time immemorial, also quinine and all the 
nervines. Recently solanine has been recommended, fifteen to 
twenty centigrammes (grs. ijss.-iijss.) a day. In my own expe- 
rience this drug does not possess much value ; neither does the 
oil of turpentine given internally in capsules containing fifteen 
minims ten or twelve times a day. In short, I consider all in- 
ternal medicines, unless the case be one of syphilis, as useless 
and inadvisable, for, owing to the long duration of the trouble, 
they would have to be taken for months with great detriment 
to the stomach and to the digestion. More is accomplished 
by external measures— massage and electricity. Both have the 
disadvantage, however, that they act very slowly and that their 
application causes more or less violent pain, a remark which 
applies more particularly to a systematic and an energetic use 
of massage (Schreiber and others). The faradic brush and the 
combined current used by De Watteville are also very painful, 
but both can be recommended with a clear conscience. With 
regard to the best manner in which the electricity should be 



376 DISEASES OF THE SPINAL NERVES. 

given, as we have said before, we now repeat that every one has 
his own method, in which he has most confidence because he 
is most familiar with it. 

If we are forced to send our patients to the springs, we may 
first of all recommend non-medicated hot springs or hot brine 
springs. Among the former may be mentioned Gastein, Her- 
kulesbad, Johannisbad, Teplitz-Schonau, and Wildbad ; among 
the latter, Wiesbaden, Nauheim, Rehme, and Baden-Baden. 
At hot sulphur springs, for instance, Landeck, Teplitz-Trenc- 
sin, and Pistyan in Hungary, such patients do very well, but 
it is advisable not to raise their expectations too high, as often 
the results of a stay at the springs are not very striking. Sea- 
bathing is not always borne well by patients with neuralgia. 
At any rate, it is well to begin with places on the Baltic and to 
select first those where warm sea-water baths can, if necessary, 
be also obtained — e. g., Colberg, Misdroy, Zoppot, and others. 
In severe cases, particularly if there occur transient attacks of 
intense pain, morphine can not be dispensed with. Subcuta- 
neous injections in proper amounts and at the proper time 
will do the patient no harm, but will afford him unspeakable 
relief, such as can be expected from no other drug. 

LITERATURE. 

Albert. Eine eigenthiimliche Art der Totalskoliose. Wiener med. Presse. i 

Marz, 1886, xxvi. 
Nicoladoni. Ueber eine Art des Zusammenhanges zwischen Ischias und Skoliose. 

Ibid., 1 886, 26, 27. 
Vinay. Paralysie radiculaire du nerf sciatique par compression a cause de l'ac- 

couchement. Revue de med., 1887, 7. 
Babinski. Sur une deformation particuliere du tronc causee par la sciatique. 

Arch, de Neurol., 1888, xv, 43, 1. 
Bernhardt. Ueber Peroneuslahmungen. Originalbericht der Gesellschaft f. 

Psych, u. Nervenkh. zu Berlin am November 12, 1888. Neurol. Centralbl., 

1888, 23. 
Schudel. Ueber Ischias scoliotica. Arch. f. klin. Chir., 1888, 38, 1. 
Weiss. Zur Therapie der Ischias. Centralbl. f. d. ges. Therap., 1889, vii, 1. 
Texier. Deformation particuliere du tronc causee par la sciatique. These de 

Paris, 1888. 
Brissaud. Des scolioses dans les nevralgies sciatiques. Arch, de Neurol., Jan., 

1890. 
Gorhan. Wiener klin. Wochenschr., 1890, 24. 

v. Bonsdorff. Finska lakaresallsk. handl, 1890, xxxii, 5. (Ischias Scoliotica.) 
Guinon et Parmentier. Arch, de Neurol., 1890, 59. 

Jasinski. Przeglad lekarski., 1890, xxix, 7, 8. (Skoliosis following Sciatica.) 
Gussenbauer. Prager med. Wochenschr., 1890, xv, 17, 18. 



LESIONS OF THE SACRAL PLEXUS. 



377 



Popper. Deutsche med. Wochenschr., 1890, 45. (Peroneal Palsy produced by- 
Mechanical Causes.) 
Remak. Alternirende Skoliose bei Ischias. Deutsche med. Wochenschr., 

1891, 7- 

Charcot, J. O., et Meige. Un cas de sciatique avec paralysie amyotrophique 
dans le domaine du poplite, determinee par l'usage exagere de la machine a 
coudre. Progres med., 1891, No. 14. 

Lamy. Deux cas de sciatique spasmodique. Progres med., 1891, 2. 

Remak. Ueber Ischias scoliotica. Deutsche med. Wochenschr., 1892, 27. 

Higier. Fiinf Falle von Ischias scoliotica. Deutsche med. Wochenschr., 

1892, 27. 

Olssen, Oscar. Ett Fall ar ischias scoliotica. Hygiea, 1892, i, iv, p. 334. 

Klamroth. Berliner klin. Wochenschr., 1892, 38, pag. 960. 

Hunermann. Arch. f. Gyn., 1892, xlii, 3, pag. 489. (Paralysis in the distribution 

of the Sciatic Nerve as a Consequence of Childbirth.) 
Sachs, W. Ein Beitrag zur Frage der Ischias scoliotica. Arch. f. klin. Chir., 

1893, xlvi, Heft 4. 

Far less frequently — we might say, only exceptionally — are 
the individual branches of the sciatic nerve the seat of neural- 
gia; thus we may have an affection of the plantar nerves, and 
sometimes the hyperesthesia in their distribution may be so 
marked that the patient is absolutely prevented from standing 
or walking. Barbillon (cf. lit.) has devoted a careful study to 
this so-called plantar hyperesthesia without, however, being 
able to decide whether the disorder is of spinal origin, or 
whether it has to be regarded as a so-called dermatalgia, or 
again as a disturbance in the nutrition of the fine nerve end- 
ings. The first explanation is supported by the fact that 
usually both feet are affected ; the last that it often occurs in 
people who have to stand a good deal. It has often been 
known to occur as a sequela of typhoid fever. Cures are said 
to have been effected by blisters, or by the application of a 
spray of methyl chloride ; bathing the feet for some time in 
hot salt solution has also been recommended. The neuralgia 
of the external plantar nerve which S. K. Morton has described 
as metatarsalgia (Annals of Surgery, June, 1893), manifests itself 
in paroxysms of pain in an area extending from the third to 
the fifth metatarso-phalangeal joint ; during these paroxysms 
the patient is unable to walk and is forced to take off his shoe. 
Badly fitting shoes and traumatism seem to be the causes of the 
affection. Sometimes it may be necessary to resect the head 
of the fourth metatarsal bone. 

The pudic nerve, which supplies the bladder, the rectum, 
the perinasum, and the external genitals, is often the seat of neu- 



378 DISEASES OF THE SPINAL NERVES. 

ralgias which are sometimes purely cutaneous and show them- 
selves by an extreme tenderness of the skin of the penis, the 
scrotum, the region of the anus, and the mons Veneris. In 
many instances the testicle is affected and, as we have pointed 
out above, becomes very tender and the seat of violent paroxys- 
mal pains. Although there may be intervals in which the neu- 
ralgia disappears, the tenderness and irritability remain as long 
as the disease of the nerves is present. 

Other nervous affections of the male urinary apparatus have 
been studied by Oberlander (cf. lit.), who has called attention 
to the fact that varicocele, chronic gonorrhoea, hydrocele mul- 
tilocularis, tuberculosis, carcinoma, etc., frequently give rise to 
such disorders, and indeed not only do the just mentioned cuta- 
neous forms occur, but also a peculiar neuralgia of the urethra, 
which becomes particularly distressing during coitus and mic- 
turition, is frequently known to develop under the influence of 
such affections. The remains of a gonorrhoea together with 
chronic dyspepsia may produce a chronic hypersesthesia of the 
mucous membrane of the bladder, to which little attention has 
been paid as yet. The pain appears periodically, affects the 
whole bladder region, and radiates into the urethra and the 
ureters. Slight errors of diet may evoke violent exacerbations 
of the trouble. Neuralgia of the bladder is found in neurasthe- 
nia, but also at times in the initial stage of tabes ; hence it would 
be necessary to decide, if we have diagnosticated a neuralgia of 
the bladder, whether it is due to a cystitis or a spinal disease, 
or whether, on the other hand, it constitutes an affection by 
itself. 

The neuralgia of the prostatic gland has recently been 
studied by Preyer of Zurich ; he distinguishes a hypersesthesia 
of the organ proper from a hypersesthesia of the prostatic por- 
tion of the urethra, and thirdly describes an irritability of the 
muscular portion of the gland. Paroxysmal pains and spasms 
of the sphincter vesicas are the most prominent symptoms of 
the affection. The treatment consists partly in attending to 
the general health, partly in surgical measures, the passing of 
sounds etc. (Berlin, Fischer, 1891). 

Anaesthesia of the mucous membrane of the bladder and of 
the urethra as well as loss of the muscular sense of the bladder 
make it impossible for the patient, to say with the eyes closed 
whether he is voiding urine or not. It may happen to tabetics, 
in whom the condition is not infrequently met with, that, hav- 



LESIONS OF THE SACRAL PLEXUS. 



3/9 



ing given up all attempts to micturate after unsuccessful strain- 
ing, they pass their urine involuntarily and become only con- 
scious of the fact when they feel the dampness of their clothes. 
This anaesthesia does not seem, however, to occur as an inde- 
pendent disease, but would appear to be always of central 
origin. 

The motor disturbances affecting the muscles which expel 
the urine and those which close the bladder may be of an irri- 
tative or a paralytic nature, the former constituting what is 
known as strangury ; the latter are by the laity comprehended 
under the name of " weakness of the bladder." Both may be 
symptoms of chronic inflammation of the urethra or of certain 
spinal diseases, and may also occur independently, as purely 
nervous affections. The desire to urinate every few minutes, 
a desire which is increased after drinking alcoholic beverages, 
is not infrequently alternated by spastic conditions of the mus- 
cles of the bulb which give rise during micturition to spas- 
modic excruciating pains in the perinaeum which radiate to the 
thighs and the buttocks. 

In all cases of this kind the treatment is generally begun with 
the usual anti-neuralgic remedies, of late years also with co- 
caine. However, the result is often very unsatisfactory. We 
should always carefully search for possible underlying abnor- 
mities, such as an elongated adherent preputium, insufficiently 
dilated or tight strictures, flexion or version of the uterus, or 
pathological changes in the rectum. If such be found the neu- 
ralgia is to be regarded as a reflex neurosis, and we have to 
direct our therapeutic efforts to the primary cause, by which 
procedure we may be able to improve and eventually cure the 
neuralgia. To the same class of reflex neuroses belongs the 
enuresis nocturna, which is rather common among children. 
The trouble can usually be traced to irritation in the urethra 
or at the orifice, such as inflammatory conditions, slight adhe- 
sions of the mucous membrane far back in the urethra, too nar- 
row an orifice of the urethra, and the like. It has been claimed 
that the urine sometimes contains an irritating substance which 
produces reflexly the enuresis, which can be controlled by the 
administration of mild narcotics. (Aqua Amygdal., araar., etc. 
— Rohde, Berl. klin. Wochenschr., 1893, 42). Here, of course, 
attention to such primary disorders is the first step in our 
treatment, and dilatation of the posterior portions of the ure- 
thra with dilators made for the purpose will often be followed 



380 DISEASES OF THE SPINAL NERVES. 

by striking results (Oberlander, Berliner klinische Wochen- 
schrift, 1888, 31). 

By coccygodinia we mean a neuralgia which is character- 
ized by pain over the region of the coccyx. The affection is 
more frequently met with in women than in men, and the pain, 
which shows paroxysmal exacerbations and comes on more par- 
ticularly during the act of defecation, may attain to a frightful 
pitch. The causes of the affection are obscure, yet we are 
probably not far from being correct in assuming that in many 
cases it is of reflex origin, as in men especially treatment of the 
genitals — a diminution of an abnormal sensitiveness of the pars 
prostatica ureth., etc. — may be followed by surprising results. 
In some cases the pains appear during sleep without any ap- 
preciable cause, in others they have been known to occur after 
traumatism. I have repeatedly observed them in neurasthenic 
and hysterical patients. The excision of the coccyx, an opera- 
tion which in desperate cases has been undertaken for relief of 
the pain, should, of course, not be resorted to until all other 
means, particularly energetic application of the faradic brush, 
have been thoroughly tried. 

LITERATURE. 

Hammond. Neuralgia of the Testicle. Neurol. Contribut, 1881, i, 3. 

Sutton. Crural Neuralgia in Dentists. Lancet, 1882, ii, 4. 

Engelhardt. Zur Genese der nervosen Symptomencomplexe bei anatomischen 

Veranderungen in den Sexualorganen. Stuttgart, Enke. 
Englisch. Ueber eine besondere Form der Hamorrhagie an den Unterextremi- 

taten (Haemorrhagia neuralgica). Wien. med. Blatter, 1885, 24-26. 
Barbillon. Hyperaesthesia plantae bilateralis. Progr. med., 1885, xiii, 19. 
Striimpell und Mobius. Ueber Steigerung der Sehnenreflexe bei Erkrankung 

peripherer Nerven. Munch, med. Wochenschr., 1886, xxxiii, 14. 
S. Laache. Norsk Magaz. f. Lagevidensk., 1886, 4 R., i, 19. (Hyperaesth. 

plantar.) 
Oberlander. Zur Kenntniss der nervosen Erkrankungen am Harnapparate des 

Mannes. Volkmann'sche Sammlung klin. Vortr., 1886, 275. 
Adamkievvicz. Ein seltener Fall von Neuralgie im N. pudendus communis mit 

gliicklichem Ausgange. Bresl. arztl. Zeitschr., 1886, 8. 
Potherat. Nevralgie vesicale. Progres med., 1887, 27. 
Nonne. Berliner klin. Wochenschr., 1887, 45. 

Hughes (St. Louis). Weekly Med. Rev., March 12, 1887. (Plantar Hyperaes- 
thesia, Neuritis N. Plantaris Interni, in Consequence of Overexertion.) 
Peyer. Zwei Falle von Neuralgie des Steissbeins bei Mannern. Centralbl. f. 

klin. Med., 1888, ix, 37. 
Bernhardt. KHn. Beitrag zur Lehre von der Innervation der Blase des Mastdarms 

und der Geschlechtsfunction. Berliner klin. Wochenschr., 1888, xxv, 32. 



LESIONS OF THE SACRAL PLEXUS. 



381 



v. Swiecicki. Zur operativen Behandlung der Coccygodynie. Wien. med. 

Presse, 1888, xxix, 31. 
Grafe, M. Zeitschr. f. Geburtsh. und Gynacologie, 1888, xv, 2, p. 344. 
Mills, Lesions of the Sacral and Lumbar Plexuses. Med. News, June 15, 1889. 

As to the motor disturbances — and of these we will take up 
first the paralyses which occur in the distribution of the sacral 
plexus — here, too, those of the sciatic nerve are the most im- 
portant. Such lesions may affect the nerve high up in the 
pelvis, or soon after its exit from it, or still lower down in its 
branches. The first are almost always caused by traumatism 
or pressure exerted for a comparatively long time — e. g., by a 
pregnant uterus, the child's head during labor (Vinav), tumors, 
etc. The others often constitute a symptom of some other 
disease. 

External popliteal (peroneal) paralysis, in which the mus- 
cles of the anterior surface of the leg are affected (the exten- 
sors of the toes, the tibialis anticus, and the peronei), is easily 
recognized. The foot hangs down flaccidly, it can not be 
dorsally flexed, abducted, nor adducted. As a result, walking 
is very much impaired, since the point of the foot often trips 
over prominences on the floor, but by raising the thigh higher 
than usual the patient somewhat overcomes the difficulty. As 
the point of the foot or the outer margin is first put to the 
ground in an awkward manner, the gait is very peculiar and 
highly characteristic of this form of paralysis. Contractures 
of the calf muscles, which may later develop secondarily, give 
rise to a permanent position of talipes equinus or talipes equino- 
varus. External popliteal paralysis may be brought on by the 
occupation of the patient. It has been seen as the result of 
pressure in those who, from the nature of their work, have to 
be constantly in a kneeling position, as, for instance, asphalt 
pavers (Bernhardt). 

Internal popliteal paralysis, which implicates the muscles of 
the back of the lower leg (the flexors, the tibialis posticus) and 
the muscles of the soles of the foot (adductor and abductor 
hallucis and the interossei), interferes with the plantar flexion 
of the foot and with flexion and lateral motion of the toes. As 
a result the patients are unable to stand on tiptoe. If the in- 
terossei take part, a condition is developed similar to that 
which is seen in the hand and which we have described on 
page 352. The toes assume a claw-like position owing to the 
fact that the first phalanx is dorsally flexed while the second 



382 



DISEASES OF THE SPINAL NERVES. 



and third are in plantar flexion. Here also secondary con- 
tractures may appear (of the tibialis anticus, triceps surae), 
which give rise to a paralytic clubfoot (pes planus, pes equinus, 
pes calcaneus). 

Paralysis of the whole sciatic makes it impossible for the 
patient to flex the lower leg on the thigh, to approach the 



N. obturator — 
M. pectineus 

M. adductor magnus 
M. adductor 



N. poplitealis externus 

M. tibialis anticus 

M. extensor longus 

digitorum 



M. peroneus 
M. peroneus brevis 

M. extensor brevis 
pollicis 



M. extensor brevis 
digitorum 




■ j M. tensor vagina: 

femoris 



M. quadriceps femoris 
M. rectus femoris 
M. crureus 
M. vastus externus 

M. vastus internus 

M. gastrocnemius 
M. solcus 



M. flexor longus 
pollicis 

M. abductor min 



digiti 



M. interossci dorsalcs 

Fig. 115.— Motor Points for the Nerves and Muscles of the Anterior Surface 

of the Leg. 



heel to the buttock, and to rotate the thigh (M. obturator in- 
ternus). Paralysis of one sciatic alone does not make walking 
absolutely impossible, because the leg fixed in the knee joint is 
moved forward by the muscles of the thigh, and so is used as a 
stilt (cf. page 226, gait of the hemiplegic). After a certain time 
muscular atrophies begin to be noticeable, and later become 



LESIONS OF THE SACRAL PLEXUS. 



38; 



very marked. The affection of the hip joint, which sometimes 
develops in the course of the paralysis, but which also at times 
has to be looked upon as the forerunner or immediate cause of 
the paralysis, produces more or less marked shortening, so that 
the patient with his affected lower extremity presents a picture 
like one of those shown in Figs. 117 and 118. This peripheral 
affection of the sciatic, which is to be regarded as a neuritis 
in the sense described above on page 331, can hardly be mis- 



Biccps femoris 
(long head) 

Biceps femoris 
{short head) 



External popliteal 
nerve 




Gluteus 



maximus 



Sciatic nerve 
Adductor magnus 
Semitendinosus 
Semimembranosus 



Internal popliteal 
nerve 



Gastrocnemius 
{external head) 

Gastrocnemius 
[interned head) 

Soleui 



Flexor communis 

digitorum 
M. flexor longus pollicis 

Posterior tibial nerve 



Fig. 116.— Motor Points for the Sciatic Nerve and the Muscles supplied by it. 



taken for anything else. The difficulty in moving one leg, 
which may amount to an actual paralysis, may, it is true, also 
be the consequence of a central cortical affection — a mono- 
plegia or monoparesis. In this case, however, the pains are 
by no means a prominent symptom, nor do we find — and this 
is the most important point of distinction — either atrophy or 
shortening. The differential diagnosis between cortical and 
peripheral paralysis has been spoken of on page 185. The 



3§4 



DISEASES OF THE SPINAL NERVE. 



treatment of the affection is to be conducted according to the 
principles which we have discussed in speaking of otherperiph- 
eral paralyses. 




Fig. ii 7 .-Case of Peripheral Neuritis of the Sciatic Nerve with Shortening 
and Atrophy of the Affected Extremity (personal observation). 

The observations which some years ago were published by West- 
phal about a periodically recurring paralysis of all four extremities 
have as yet no practical importance, since we do not know anything 
about its nature. The same may be said about the peculiar paresis 
of the lower leg and foot which Zenker has described (Berliner klin- 
ischer Wochenschrift, October 8, 1883), and which has to be regarded 
as an occupation neurosis. It occurs not rarely in persons who have 
to remain a long time in a kneeling or squatting position, and such 
instances have been known to occur in potato pickers. It manifests 
itself in a more or less pronounced sensory or motor paralysis of the 
lower part of one or both lower extremities. 



LESIONS OF THE SACRAL PLEXUS. 



385 



Spasms in these muscles are rare and are therefore of but 
little practical importance ; a case of tic convulsif in the ilio- 
psoas has been described by Klemperer (Deutsche Med.-Ztg., 
1890, 86). Bernhardt has described a case in which there 
were spasms in the region of the N. peron. dext. superfic, with 




Fig. 118.— Case of Peripheral Neuritis of the Sciatic Nerve with Shortening 
and Atrophy of the Affected Extremity (personal observation). 

clonic twitchings in the peroneus longus and brevis (Berl. klin. 
Wochenschr., 1893, 17). Schultze has described spastic con- 
ditions in the tensor fasciae latae (Deutsche Zeitschr. f. Nerven- 
heilk., 1892, iii). Spasmodic tonic contraction of the hip mus- 
cles has been described by Stromeyer as spastic contraction of 
the hip. A case of spasm confined to the quadratus lumborum 
has come under my notice in an hysterical woman. It is illus- 
trated in Figs. 119 and 120. Tonic spasm of the quadriceps 
25 



3 86 



DISEASES OF THE SPINAL NERVES. 



gives rise to extension of the leg in the knee joint ; it is some- 
times known to occur in neuralgias of the joint. The very 
painful cramp in the calf muscles, which sometimes occurs 
after great exertion, sometimes also in the course of certain 
grave general diseases — for example, cholera— is well known. 





Fig. 119. Fig. 120. 

Figs. 119, 120. — Contracture in the Quadratus Lumborum (personal observation)- 

Clonic spasms of the muscles of the lower extremities may 
be observed in hysterical patients. The so-called " saltatory 
spasm " (Bamberger, Wiener medicinische Wochenschrift, May 
4, 1859), which forces the patients whenever their feet touch 
the ground to jump, is not an independent affection, but only a 
symptom of central disease. The increase of the reflexes, 
which is generally present, is in favor of this view. Of the 
treatment we shall speak in the chapter on Hysteria. 



MULTIPLE NEURITIS. 387 

LITERATURE. 

Guttmann. Fall von sogenannten saltatorischen Krampfen. Berliner klin. 

Wochenschrift, 1867, iv, 13. 
Frey. Ueber saltatorischen Reflexkrampf. Arch. f. Psych, u. Nervenhk., 1875, 

vi, 1. 
Kast. Ueber saltatorischen Reflexkrampf. Neurol. Centralbl., 1883, ii, 14. 
Kollmann. Deutsche med. Wochenschr., 1883, ix, 40. 

V. Neuritis involving Several Spinal Nerves at the Same Time — 
Multiple Neuritis — Polyneuritis. 

Just as we have seen that several of the cranial nerves can 
be affected at the same time, so none the less is this true of the 
spinal nerves. It is, however, not many years since it has been 
shown that such multiple nerve affections may occur primarily, 
that they are often of an inflammatory nature, that they give 
rise to numerous symptoms which may, under certain circum- 
stances, be misinterpreted, inasmuch as they may simulate 
those of central lesions. The affection is known as multiple 
neuritis, and, as we said, our knowledge of it is of quite recent 
date (Dumenil, Eisenlohr, Leyden, Strumpell, Vierordt, and 
others). We may confidently expect that in the near future 
we shall obtain further information upon certain points in con- 
nection with this disease which have not as yet been cleared up. 

As we have above, on page 331, devoted some time to the 
description of the anatomical features of the disease, it remains 
for us here to speak first of the symptoms of multiple neuritis. 
It is remarkable to note that the onset frequently resembles 
that of an acute infectious disease : there are fever, general 
malaise, dull headache, apathy, etc. ; soon pains make their 
appearance, first in the lumbar region and the back, then in 
the course of the large nerve trunks. These are followed by 
an impairment of mobility, especially in the lower extremities, 
which makes the patient very anxious ; the legs are heavy, 
they are moved only by a strong effort, and not without pain, 
and the patient is easily fatigued. The reflexes are diminished 
or lost, electrical excitability is decreased, but the pains — and 
this should be emphasized — usually soon abate and other 
sensory disturbances, paresthesias and anaesthesias, are only 
exceptionally met with (Barrs, Amer. Journ. Med. Sc, Febru- 
ary, 1889), the disorder chiefly affecting the motor apparatus. 
Repeatedly cases have been observed in which the motor 
disturbances made their appearance quite suddenly, an onset 



388 DISEASES OF THE SPINAL NERVES. 

which we could almost call apoplectiform. Without any pre- 
monitory symptoms there come on violent radiating pains, 
with motor paralysis. Sometimes we find atrophy in certain 
groups of muscles ; reaction of degeneration can soon after be 
demonstrated ; sometimes thickening and a considerable in- 
crease in the subcutaneous tissue develop. If this takes place 
in the palm of the hand we have the "flat-hand," in which the 
normal hollow is absent, a condition analogous to that of " flat- 
foot " (Lowenfeld, 2 Falle neuritischer " Platt-hand," Miinchener 
med. Wochenschr., 1889, 24). Besides muscular atrophy we 
may find ataxia, and this symptom may indeed be very marked, 
so that it dominates the whole picture and makes it resemble 
that of tabes. In such cases the term pseudo-tabes peripherica, 
instead of simply multiple neuritis or polyneuritis, is very ap- 
propriate. 

If the pains are very intense, and if we find more or less 
well marked swellings, while other sensory disorders are only 
slight, the case may be one of acute primary polymyositis, a 
condition which has been well described by Strumpell. This 
is especially likely to be the case if the pains are localized in 
certain muscles (Deutsche Zeitschr. f. Nervenhk., i, 5, 6). Lewy 
has also furnished some important practical contributions to our 
knowledge of this disease (Berlin, klin. Wochenschr., 1893, 18). 

No description of the course of the disease which would 
fit all cases is possible, because this varies and presents pecul- 
iarities according to the pathogenesis. Dejerine has described 
a case of haemorrhage in the region of the brachial plexus which 
was followed immediately by paralysis of the arm (Compt. 
rend, hebdom. des seances de la Soc. de Biol., 1890, No. 27); 
but such a sudden onset is exceptional. If a multiple neuritis 
occurs in the course of another disease, its manifestations are 
not the same as when it is a primary affection, which has de- 
veloped under the influence of some special cause. Among 
the conditions in which polyneuritis may develop we would 
mention phthisis pulmonalis, diabetes (Charcot, Arch, de Neu- 
rologie, Mai, 1890, xix, 57), tabes, articular and muscular rheu- 
matism, polyarthritis, and finally the puerperal state (Desnos, 
Pinard, et Joffroy, l'Union med., 1889, 14). It has repeatedly 
been described as a sequela of typhoid fever, of small-pox, of 
scarlet fever, of diphtheria, of carcinoma (Auche, Revue de 
med., 1890, x, 10), and of leprosy (Arning und Nonne, Virch. 
Arch., 1893, cxxxiv, Heft 2) — "infectious form" of Leyden. 



MULTIPLE NEURITIS. 



389 



As an independent disease it may be caused by overexertion. 
Two cases which we have described were due to prolonged 
work with the sewing machine (cf. lit.). It may also appear, 
and this is unquestionably much more common, as a conse- 
quence of the action of certain poisons, more especially alco- 
hol, nitrobenzine, aniline (Ross and Bury), carbon monoxide, 
bisulphide of carbon, lead, arsenic, and mercury — the " toxic 
form " of Leyden. Besides these two there is, according to 
Leyden, a third variety, the so-called atrophic (anaemic, ca- 
chectic) form, which develops after a long and severe sickness, 
somewhat in the manner recently described by Oppenheim 
and Siemerling. 




Fig. 121. — Atrophy of the Muscles of the Right Upper Arm in Consequence of 
a Fracture of the Humerus Seven Years previously (personal observation). 

Sometimes sensory, sometimes motor disturbances are the 
predominating symptoms. In the neuritis of phthisical pa- 
tients both are marked to about the same extent. Occasionally 
certain nerves seem to be more liable to suffer — for instance, 
according to Mobius, during the puerperal state, the median 
and ulnar, the terminal branches of which are affected either in 
both hands or only in the one which is used more extensively, 



390 



DISEASES OF THE SPINAL NERVES. 



as a rule the right. In tabes, on the other hand, no region 
seems to be exempt, and, as Oppenheim, Siemerling, Pitres, 
Vaillard, and others have observed, not only the peripheral 
spinal, but also the cranial nerves may be attacked by the 
neuritis — for example, the vagus and its laryngeal branches, 
and the ocular nerves. Korsakow and Serbski have described 




\ 




Fig. 122.— Panarthritis with Secondary Multiple Neuritis. 

the mental symptoms which may be associated with multiple 
neuritis (Arch. f. Psych, und Nervenk., 1891, xxiii, 1, p. 112). 

The neuritis which occurs in the course of joint affections 
often leads to considerable atrophy in those muscles which are 
supplied by the affected nerve twigs. Chronic inflammation of 
the synovial membranes caused by sprains, chronic inflamma- 
tions of joints, articular rheumatism, frequent attacks of gout, 



MULTIPLE NEURITIS. 



391 



traumatism, fractures which give rise to some impediment in 
the circulation — all these causes may bring about extensive mus- 
cular atrophies. A case to the point is illustrated in Fig. 121 ; 
the patient was a boy, fifteen years old, who had sustained a 
fracture of the upper arm when he was eight years old. The 
fracture healed slowly, and was followed by atrophy of the 





Fig. 123.— Panarthritis with Secondary Multiple Neuritis. 

right upper arm and the muscles of the chest. References 
bearing on these affections and upon " reflex atrophies," which 
we shall soon mention, will be found on page 396. The case 
which we have illustrated in Figs. 122 and 123 was that of a 
young man who suffered from a panarthritis, and who in con- 
sequence of his joint affection developed muscular atrophy in 
all four extremities, more especially in the upper arms and 



39 2 



DISEASES OF THE SPINAL NERVES. 



thighs. The hip and shoulder joints, as well as the knee and 
elbow joints, had been swollen and painful for years. That 
this atrophy, which may be due to an inflammation of the fine 
end twigs of the nerves, may also be caused reflexly by the 
joint affection has been shown by Charcot. If the hip joint is 
attacked, the flattening of the buttock, the abnormally high 
position of the gluteal fold, the marked prominence of the tro- 
chanter on that side, are striking features. If the upper ex- 
tremities, especially the hands, are the seat of the disturbance, 
the atrophy gives rise to deformities which are either of the 
extensor or the flexor type (Charcot). 

Peculiar and manifold are the manifestations of that variety 
of neuritis which is produced by the abuse of alcohol. For 
the sake of simplicity we may distinguish two cardinal forms 
of this affection, although the clinical pictures of the two can 
often not be well separated from each other. In the first the 
motor disturbances and the atrophies, in the second the sensory 
disorders, are the prominent symptoms. In the former case 
the patients complain of violent tearing and drawing pains in 
the lower, more rarely in the upper extremities, which are rela- 
tively rapidly followed by a marked difficulty in walking. The 
gait of the patient is distinctly ataxic and resembles most 
closely that of a tabetic, with the exception that in the latter 
no diminution in the strength of the muscles can be noted, 
while in alcoholic neuritis it can undoubtedly be demonstrated 
and is to be explained by the muscular atrophy which occurs 
comparatively early and which is particularly seen in the ex- 
tensors. The degree to which walking in particular and mo- 
tion in general is interfered with is very variable. Sometimes 
the patient can hardly raise himself in bed without assistance, 
sometimes he may for months be able to get about fairly well 
without help. It is interesting to note that the patellar reflex 
is lost very early and completely, a circumstance which may 
lure not the inexperienced alone into making a diagnosis of 
tabes dorsalis. This is still more likely to occur, and the mis- 
take is more excusable, if the action of the alcohol has also 
manifested itself on the ocular nerves, so that, e. g., we may, in 
addition to the symptoms mentioned, encounter a paralysis 
of the abducens, which I have myself seen several times in 
alcoholic neuritis, and which Suckling (cf. lit.) and others have 
described ; or, again, the oculo-motor may be affected and 
the patient may complain of diplopia. Pierson, Eisenlohr, 



M UL TIPLE NE URI PIS. 



393 



Striimpell, and others have reported cases in which the facial 
nerve was implicated. Vagus neuroses have been reported 
in this connection, especially tachycardia, by Dejerine. If we 
add to this the frequency with which Romberg's sign (swaying 
while standing with the heels and toes together and eyes 
closed, in consequence of the disturbance of the muscular 
sense) is found in the disease, if we remember that stomach 
symptoms occur in both affections — in alcoholism as vomitus 
matutinus in consequence of a chronic gastritis, in tabes as gas- 
tric crises in consequence of disease of the vagus nucleus — we 
can not be surprised at the frequency with which alcoholic 
paralysis is taken for tabes. Nevertheless, it is not so difficult 
to avoid such a mistake, more especially if we have a chance to 
examine the patient repeatedly and do it carefully enough. 
We should particularly note the condition of the pupils. The 
absence of the Argyll-Robertson sign and the absence of 
bladder symptoms, both of which are very common in tabes, 
will be significant features. In alcoholic neuritis, further, the 
nerve trunks are usually painful and the course of the disease 
differs in the two maladies. In tabes, as we know, the outcome 
is very unfavorable, while in alcoholic neuritis, if the cause is 
removed, it is usually good. Even the individual symptoms 
may, if analyzed carefully, give us some valuable diagnostic 
hints. For example, it will hardly be very difficult for the care- 
ful examiner to distinguish the morning vomiting of alcoholics 
from the paroxysmal spontaneous vomiting of tabes, which 
appears now and again and may not reappear for months. 

The second form of alcoholic neuritis may run its course 
without giving rise to any decided motor disturbances. The 
patient then only complains of pains which sometimes run along 
the nerve trunks, becoming very violent, and may resemble the 
lancinating pains of tabes. He may complain of localized hy- 
peraesthesias and anaesthesias, of formication and numbness, all 
of which symptoms are especially marked in the lower extremi- 
ties. Various vaso-motor and trophic disturbances are not 
uncommon. CEdema may occur and disappear again, skin 
eruptions, perforating ulcers (Helbing, Beitrage zur klin. Chi- 
rurgie, 1889, v, 2), circumscribed areas of an hyperidrosis, and 
ichthyotic changes of the epidermis (Eulenburg) may be noted. 
Brissaud has published studies upon the influence of the 
trophic centres, especially in toxic neuritis (Arch, de Neur.. 
1891, xxi, 62). 



394 



DISEASES OF THE SPINAL NERVES. 



In all cases the psychical condition ought to be carefully 
considered. It may present changes very early in the disease. 
Thus Oppenheim has reported instances in which the alcoholic 
neuritis occurred simultaneously with delirium tremens. 

It has long been known that neuritis may be produced arti- 
ficially, and that it, for example, often occurs as a consequence 
of subcutaneous injections of ether ; but this has only been 
carefully studied of late years. Cases of this kind impress 
upon us the necessity of being cautious in giving the injections 
for therapeutic purposes and of avoiding especially a too deep 
insertion of the needle where we should be liable to strike 
branches of the musculo-spiral or other nerves. Paralysis of 
the extensors of the ringers has been relatively often observed. 
References bearing upon this subject will be found at the end 
of this chapter. 

In the treatment of neuritis our first aim should be to re- 
move the cause ; only when this is possible can w T e hope for 
permanent results. The therapeutic measures differ according 
as the case is recent or of old standing. If the former is the 
case, the salicylates, antipyrin, phenacetin, and, if the pains are 
very intense, morphia are indicated. According to our experi- 
ence, inunctions are of comparatively little value ; nevertheless 
an ointment containing chloroform, veratrin, and morphia may 
be tried. Wet packs (Priessnitz bandages) are sometimes serv- 
iceable, and warm baths (at 90 to 95 ) may be beneficial, but 
cold water is usually dreaded by the patients. The most 
important measure in these cases is the electrical treatment. 
Where this can not be used, or where it can not be properly 
applied, it is impossible to do much, and it is then best to leave 
the case to Nature, a course which frequently results in re- 
covery, though this is apt to be slow. The constant and the 
combined current (De Watteville) should be used somewhat in 
the manner described for the treatment for the motor nerves 
in my Text-Book on Electro-Diagnosis and Electro-Therapeutics 
(Stuttgart, Enke, 1893, pp. 142, 143). Next to the correct ap- 
plication of electricity, the most important point to remember 
in this treatment is that we must not give it up too soon, and 
that we should not despair if at first no results can be seen. 
Several weeks, even two or three months, will be necessary in 
any grave case. Sometimes even the protracted use of elec- 
tricity has no effect, and we may well say that the treatment of 
multiple neuritis is rarely a grateful task. 



MULTIPLE NEURITIS. 



LITERATURE. 



395 



a. Multiple A r eztritis. 
Striimpell. Zur Kenntniss der multiplen degenerativen Neuritis. Arch. f. 

Psych, u. Nervenkrankh., 1883, xiv, 2. 
Hirt. Beitrag zur Pathologie der multiplen Neuritis. Neurol. Centralbl., 1884, 21. 
Oppenheim. Multiple Neuritis. Deutsches Arch. f. klin. Med., 1885, Bel. 36, 

Heft 5, 6, p. 561. 
Buzzard. Paralysis dependent upon Peripher. Neuritis. Lancet, November 28, 

December 12, 1885. 
Pitres et Vaillard. Peripheral Neuritis in Tuberculosis. Revue de med., 1886, 

No. 3. (Latent or Associated with Atrophy or Sensory Disturbances.) 
Francotte. Nevrite multiple. Revue de med., 1886, No. 5. 
Oppenheim. Ueber interstitielle Neuritis, ihr Vorkommen bei Nerven- unci 

anderen Erkrankungen. Neurol. Centralbl., 1886, No. 11, pp. 255 et seq. 
Massalongo. Le neuriti multiple periferiche primitive e specialmente della forma 

di polineurite acuta. Gaz. degli ospitali, 1886, Nos. 55, 56, 58-62. 
Pitres et Vaillard. Nevrites peripheriques dans le rhumatisme chronique. Re- 
vue de med., 1887, viii, 6, p. 456. 
Grimodie. Contribution a l'etude de la pathogenie des nevrites peripheriques. 

These de Paris, 1887. 
Mobius. Ueber Neuritis puerperalis. Miinchener med. Wochenschr., 1887, No. 

9. (Attacks most frequently the ulnar and the median nerve.) 
Stadelmann. Neurol. Centralbl., 1887, 17. (Peculiar Condition found in a Case 

of Neuritis of the Brachial Plexus coming on after Typhoid.) 
Goldflam. On the so-called Multiple Neuritis. Medycyna, 1887, xv, 23-28. 
Oppenheim und Siemerling. Beitrage zur Pathologie der Tabes dorsalis und 

der peripherischen Nervenerkrankungen. Arch. f. Psych, und Nervenkh., 

1887, xviii, 1, 2. 
Dubois. Ueber apoplectiformes Einsetzen neuritischer Lahmungen. Corre- 

spondenzbl. f. Schweiz. Aerzte, 1888, 14. 
Senator. Ueber acute multiple Myositis bei Neuritis. Deutsche med. Wo- 
chenschr., 1888, xiv, 23. 
Goldflam. Zeitschr. f. klin. Med., 1888, xiv, 4. 
Bury. Peripheral Neuritis in Acute Rheumatism and the Relation of Muscular 

Atrophy to Affections of the Joints. From the Medical Chronicle, June, 

1888. 
Klumpke. Des polynevrites en general et des paralysies et atrophies saturnines 

en particulier. Paris, F. Alcan, 1889. 
Dehio (Dorpat). Ueber die Erkrankungen der peripheren Nerven bei Lepra. 

Petersburger med. Wochenschr., 1889, 42. 
Mobius. Beitrag zur Lehre von der Neuritis puerperalis. Miinchener med. 

Wochenschr., 1890, 14. 
Feilchenfeld. Neuritis multiplex cum glycosuria. Deutsche med. Wochenschr., 

1890, 19. * 

Bruns. Ueber neuritische Lahmungen beim Diabetes mellitus. (" Diabetische 

Lahmungen.") Berliner klin. Wochenschr., 1890, 23. 
Frankel, A. Ueber multiple Neuritis. Deutsche med. Wochenschr., 1891, 53. 
Pal. Ueber multiple Neuritis. Wien, Holder, 189 1. 



396 DISEASES OF THE SPINAL NERVES. 

Lloyd. Forms of Pseudo-tabes due to Lead, Alcohol, Diphtheria, etc. Med. 
News, 1892, 14. 

Engel-Reimers. Beitrage zur Kenntniss der gonorrhoischen Nerven- und 
Ruckenmarkserkrankungen. Jahrbiicher der Hamburger Staats-Kran- 
kenanstalt, 1892. 

Leyden. Ueber Polyneuritis mercurialis. Deutsche med. Wochenschr., 1893, 31. 
(Mercurial Treatment of Syphilis.) 

Ross and S. Bury. On Peripheral Neuritis. London, Griffin & Co., 1893. 

Giese und Pagenstecher. Beitrag zur Lehre von der Polyneuritis. Arch. f. 
Psych, und Nervenkrankh., 1893, xxv, 1, p. 211. 

Mills, Charles K. Neuritis and Myelitis and the Forms of Paralysis and Pseudo- 
paralysis following Labor. University Med. Magazine, May, 1893. 

b. Alcoholic Neuritis. 

Fischer. Ueber eine eigenthiimliche Spinalerkrankung b. Trinkern. Arch. f. 

Psych., 1882. 
Dreschfeld. Brain, July, 1884, p. 200. (Chronic Alcoholism : Ataxia in Men, 

Atrophies in Women.) 
Broadbent. On a Form of Alcoholic Spinal Paralysis. Med.-Chir. Transact., 

vol. lxvii. 
Charcot. Les paralysies alcooliques. Gaz. des hop., 1884, No. 99. 
Kruche. Die Pseudotabes der Alkoholiker. Deutsche Med.-Ztg., 1884, No. 72. 
Moeli. Statist, u. Klin, liber Alkoholismus. Charite-Annalen, 1884, ix, p. 524. 
Schulz. Neuritis der Potatoren. Neurol. Centralbl., 1885, Nos. 19, 20. 
Hadden. Cases illustrating the Symptoms of Chronic Alcoholism. Lancet, 

October 3, 1885, p. 610. (Hyperaesthesia of the Skin, Vomiting, no Patellar 

Reflex, Plantar Reflex retained.) 
Bernhardt. Ueber die multiple Neuritis der Alkoholisten. Zeitschr. f. klim 

Med., 1886, xi. 
Brissaud. Des paralysies toxiques. These d'agregation de Paris, 1886. 
Oettinger. Etude sur les paralysies alcooliqties. These de Paris, 1885. 
Dejerine. Contribution a l'etude de la nevrite alcoolique. Arch, de Phys., 1887, 

x, 5me ser., p. 248. 
Witkowski. Zur Kenntniss der multiplen Alkoholneuritis. Arch. f. Psych, u. 

Nervenkrankh., 1887, xviii, 3, p. 809. 
Bonnet. Arch, de neurologic Juillet, 1887, pp. 79 et seq. 
Suckling. Ophthalmoplegia externa due to Alcohol. Brit. Med. journ., March 

3, 1888. 
Eichhorst. Neuritis fascians alcoholica. Virchow's Archiv, 1888, 112, 2. 
Siemerling. Kurze Bemerkungen zu der von Eichhorst sogenannten Neuritis 

fascians. Arch. f. Psych., 1888, xix, 3. 
Guillemin. Annales Med. -Psych., Mars, 1888, 7me ser., 2. (Alcoholic Hysteria.) 
Wladar, Martin. Wiener med. Presse, 1888, xxix, 10. (Angioneurosis of the 

Vessels of the Head as a Result of Alcohol and Nicotine Intoxication.) 
Sharkey. Alcoholic Paralysis of the Phrenic, Pneumogastric, and other Nerves. 

Transactions of the Pathol. Society, 1888, xxxix, p. 27. 
Schaffer. Neurol. Centralbl., 1889, viii, 6. 
Siemerling. Charit6-Annalen, 1889, xiv, p. 443. 
Buzzard. Brit. Med. Journal, June 21, 1890. 



DISEASES OF THE TROPHIC AND VA SO-MOTOR NERVES. 



397 



c. Neuritis caused by Subcutaneous Injection of Ether. 

Salvat. These inaug., Bordeaux, 1884. 

Remak und Mendel. Berliner klin. Wochenschr., 1885, xxii, 5, pp. 76, 77. 

Hadra. Sitzung der Bed. med. Gesellsch., v, Juni 3, 1885. 

Pitres et Vaillard. Des nevrites provoquees par les injections d'ether ail voisi- 

nage des troncs nerveux des membres. Gaz. med. de Paris, Mai 28, 1887, 

No. 22. 

d. Muscular Atrophy after Joint and Bone Disease — "Reflex Atrophy" {Charcot). 

Charcot. Prog, med., Juin-Juillet, 1882. 

Mondan. Recherches experimentales et cliniques sur les atrophies des mem- 
bres. Valence, 1882. 

Deschamps. Contribution a l'etude des atrophies musculaires a distance, appel- 
lees encore, "atrophies reflexes." These de Paris, 1883. (The trophic 
force of the nerve centres is diminished according to this author.) 

Cornillon. Progr. med., 1883, xi, 21, p. 405. (Muscular Atrophy after Attacks 
of Gout simulating Progressive Muscular Atrophy.) 

Striimpell. Munch, med. Wochenschr., 1888, 13. (Muscular Atrophy after 
Acute Articular Rheumatism.) 

Wichman. Der chron. Gelenkrheumatismus und seine Beziehungen zum 
Nervensystem. Neuwied, 1890. 

Raymond. Recherches experimentales sur la pathogenie des atrophies muscu- 
laires consecutives aux arthrites traumatiques. Revue de med., 1890, x, 5. 

Dubreuilh. Myopathies nevritiques. Ibid., 1890, 6. 

Darkschewitsch. Atroph. muse, arthropath. Neurol. Centralbl., 1891, 12. 

Lavieille. Sur une arthrite speciale du pied avec deformation observee chez les 
velocipedistes. Paris, 1891. 

Duplay et Cazin. Arch. gen. de med., 1892, 1. (Muscular Atrophy after Joint 
Disease.) 

Hugh, Lane. Deutsche Med.-Ztg., 1893, 19. (The Neuroses in Chronic Rheu- 
matoid Arthritis.) 

Charcot. Amyotrophies speciales reflexes d'origine articulaire. Progres med., 
i893. 13- 

B. Diseases of the Trophic and Vaso-motor Nerves. 

In spite of the epoch-making labors of Samuel (cf. lit.), who, 
after Romberg, was the first to postulate the existence of defi- 
nite " trophic " nerve fibres for the regulation of the nutrition 
of the tissues, we are to-day still unable to demonstrate such 
fibres, nor do we know whether there exist purely trophic 
centres, or whether the trophic influence is exerted by some 
centres already well known — viz., by the motor, sensory, or 
vaso-motor. On the other hand, the existence of such a direct 
trophic influence of the nervous system upon the tissues can 
not be called in question. Again, we can not as yet decide 
whether or not this influence, upon which the nutrition of the 



398 DISEASES OF THE SPINAL NERVES. 

tissues normally depends, is different for different tissues — as 
indeed seems quite probable — and whether it has to be re- 
garded as acting by inhibition or rather by an active stimu- 
lation. 

Occasionally, besides the trophic we meet also with vaso- 
motor disturbances, which lead us to conclude that not only 
the vaso-constrictor but also the less important vaso-dilator 
nerves may become affected. With their anatomical relations 
we are better acquainted than with, those of the trophic nerves. 
At any rate, we know that in the cerebrum and in the medulla 
oblongata vaso-motor centres do exist. In their further course 
the vaso-motor fibres are thought to pass through the lateral 
columns of the spinal cord and to leave it through the anterior 
roots, but this is not yet proven ; nor can we accept the exist- 
ence of Goltz's vaso-motor reflex centres in the spinal cord 
without further investigation. 

The vaso-motor disturbances which we sometimes see in 
acute diseases — for instance, in typhoid fever (Money, Lancet, 
December 3, 1887) — are phenomena due either to a condition 
of paralysis or of irritation. In the former case we have red- 
dening of the skin with elevation of the temperature and more 
or less pronounced pain, which become more marked when the 
patient is exposed to a warmer atmosphere, and which are not 
with our present resources amenable to treatment. This affec- 
tion, which has been attributed to a diminished energy of vas- 
cular innervation, has recently been called " erythromelalgia " 
(Weir Mitchell). In the latter we have marked pallor and 
coldness of the skin, associated with formication, subjective 
sensations of cold, as, for example, in the so-called anaesthe- 
sia lavatricum (cf. Hirt, Krankheiten der Arbeiter, 1878, Part 
II, p. 100). Such disorders do not, however, always lead to 
trophic changes. It is rather probable, as Kopp thinks, that, 
if the latter occur, disturbances in the nutrition of the vessel 
walls must have preceded them (cf. the work of Thoma, lit.). 

Sometimes vaso-motor and trophic disturbances may co- 
exist as genuine complications. 

Of clinical importance is the fact that trophic changes may 
occur either by themselves or accompany other central, cere- 
bral, as well as spinal affections. This may be explained by the 
fact that, in order to bring about alterations in the trophic influ- 
ences, it is not necessary to have a disease of the ganglionic 
cells or cell groups, which probably act as centres, but that 



DISEASES OF THE TROPHIC AND VA SO-MOTOR NERVES. 3™ 

pathological processes in the peripheral nerves may also have 
the same effect. Among the central affections, which, how- 
ever, may remain latent for a long time, so that one might be 
led to regard the trophic changes as independent affections, we 
must mention in the first place tabes — which we shall discuss in 
this connection later — hysteria, certain cerebral diseases due to 
changes in the vessels, such as apoplexy with the acute bedsore, 
of which we have spoken on page 232, and again diseases of 
the gray axis of the spinal cord (Jarisch), among others the 
" paralysie generale spinale anterieure subaigue " (Pitres et 
Vaillard, Prog, med., 1888, 35). To the diseases of the periph- 
eral nerves, and the infectious diseases in the course of which 
trophic disturbances may occur, w r e have already alluded. 

At present we can form no idea how many diseases, not 
only of the nerves and of the muscles but also of other organs, 
we shall have to call " trophic " when we have once become 
better acquainted with the position of the trophic centres and 
fibres than we are now. For the present the term is restricted 
to a small number of affections, and it will suffice to say a few 
words about the most important among them, and first about 
the tropho-neuroses of the skin. 

Anomalies of secretion which have to do with the sebaceous 
as well as the sweat glands are not uncommon. It is well 
known that seborrhcea, for example, may occur after long- 
standing menstrual disturbances, chlorosis, ansemia, after over- 
exertion, or as a consequence of too great sexual excitement, 
masturbation, etc., especially in young individuals, whereas 
diminished secretion of the sebaceous glands, as found, for in- 
stance, in ichthyosis and in senile atrophy of the skin, is com- 
paratively rare. The purely nervous origin of this, as well as 
of hyperidrosis and anidrosis, can hardly be questioned. Hy- 
peridrosis is seen on one side alone or on both sides in central 
diseases — for instance, in some diseases of the medulla oblon- 
gata (Traube), of the spinal cord (spinal apoplexy, myelitis), and 
of the entire nervous system (tabes, hysteria). It also occurs 
reflexly .(Raymond). The anidrosis appears in peripheral facial 
paralysis, in dementia paralytica, and in certain skin affections, 
such as psoriasis, lichen, and ichthyosis. 

Among the skin affections associated with exudation we 
have erythema nodosum, urticaria, and a disease probably akin 
to it, the angio-neurotic oedema (Quincke), which appears some- 
times quite suddenly on different parts of the body, the patient 



400 DISEASES OF THE SPINAL NERVES. 

feeling otherwise perfectly well. The hydrarthrosis intermit- 
tens, which Fere has regarded as an articular angio-neurosis 
(Revue de Neurol., 1893, 17), and cutaneous swellings of nerv- 
ous origin accompanying the menses (E. Boerner, Volkm. Samml. 
klin. Vortr., 1888, xi, No. 312), have been described. Again, 
we have certain forms of eczema, prurigo, herpes zoster, and 
others, although their nervous origin is not established beyond 
doubt. As every one of these affections presents in its devel- 
opment, in its clinical significance, and in its treatment, so much 
that is by no means clear, we deem ourselves hardly called 
upon to enter into a detailed description of them here. Some, 
as, for instance, the herpes zoster in the course of facial paraly- 
sis, have been mentioned above (cf. page 90). Equally obscure 
is the origin of cutaneous haemorrhages — as, for instance, the 
ecchymoses which occur in tabes after severe attacks of 
pain — of the pigment hypertrophies (e. g., in lepra), of the 
anomalies of cornification (keratosis and ichthyosis), of the 
naevus, which is said to be due to intra-uterine disease of the 
spinal ganglia, of the atrophic conditions of the skin (striae and 
maculae atrophicae), of the so-called glossy skin (glossy fingers), 
of the pigment atrophies (vitiligo), of the atrophy of the hair, 
and the atrophies or deformities of the nails, changes which we 
meet with in the most varied nervous affections and under the 
most varied circumstances. 

An interesting angio-neurosis is the so-called night palsy, 
which has been described by Ormerod, Bernhardt, and others. 
It consists in numbness, pain, and a feeling of weakness occur- 
ring at night in the upper extremities. Distinct anaesthesia and 
actual paralysis are not present. Women are affected more 
frequently than men, and seem to be particularly prone to it at 
the menopause. 

LITERATURE. 

Samuel. Die trophischen Nerven. Leipzig, i860. 

Lustig. Zur Lehre von den vasomotorischen Neurosen. Inaug.-Diss., Breslau, 
1875. 

Alexander. Lancet, 1881, i, 25, 26. 

Stiller. Wiener med. Wochenschr., 1881, 5, 6. 

Seeligmiiller. Ueber Hydrops articulorum intermittens. Deutsche med. Wo- 
chenschr., 1880, 5, 6. (Is by Seeligmiiller regarded as a vaso-motor 
neurosis.) 

Schwimmer. Die neuropathischen Dermatosen. Wien u. Leipzig, 1883. 

Weiss. Prager Zeitschr. f. Heilk., September 15, 1885, vi, 6. (Zoster cerebralis.) 

Kopp. Die Trophoneurosen der Haut. Wien, 1886, Braumiiller. 



RA YNA UD'S DISEA SE. 



40 1 



Renault. Note relative des troubles trophiques exceptionels d'origine rhumatis- 

male. Gaz. hebd., 1887, xliii, 24. 
Raymond. Des ephidroses de la face. Arch, de neurol., 1888, 43, p. 51. 
Thoma. Ueber das Verhalten der Arterien bei Supraorbitalneuralgie. Deut- 

sches Arch. f. klin. Med., 1888, Bd. xliii, Heft 4, 5. 
Seguin. Boston Med. and Surg. Journ., October, 1888, cxix, 15. 
Auche et Lespinasse. Cas d'Erythromelalgie. Revue de med., 1889, 12. 
Scheiber. Falle von Trophoneurosis. Review in Wiener med. Presse, 1890, 27. 
Josef, M. Berliner klin. Wochenschr., 1890, 4, 5. 
Griitzner, P. Einige neuere Arbeiten liber trophische Nerven. Deutsche med. 

Wochenschr., 1893, 1. 
Bauke. Zur Aetiologie des " acuten angioneurotischen " oder "umschriebenen 

Hautodems." Berliner klin. Wochenschr., 1892, 6. 
Gerhardt. Ueber Erythromelalgie. Deutsche med. Wochenschr., 1892,39. 
Diinges. Der praktische Arzt, 1893, 10. (Case of Erythromelalgia.) 
Pojor. Fall von Erythromelalgie. Pester med.-chir. Presse, 1893. 
Eulenburg. Ueber Erythromelalgie. Deutsche med. Wochenschr., 1893, 50. 
Lewin und Benda. Ueber Erythromelalgie. Berliner klin. Wochenschr., 1894, 3. 

The so-called symmetrical gangrene of the fingers and toes 
(sclerodactyly) which was first described in 1882 by Raynaud, 
and which has, after him, been called Raynaud's disease, comes 
on with the following symptoms : The fingers appear at times 
as if dead (" doigts de mort "), at another time they turn a dark- 
red color and burn violently. Gradually disturbances in nu- 
trition, at first only transitory, later permanent, develop, and 
blebs form, which open, leaving a sore which heals with loss 
of substance. The nails fall out and are not replaced, whole 
parts die, the necrosis being symmetrical on both sides, and 
none of the usual causes of gangrene — such as disease of the 
heart or of the blood-vessels, septicaemia, traumatism, etc. — 
are present. The disease is, however, very rarely met with in 
its full development, while lighter grades, in which we have 
only to deal with a transient spasm (or paralysis) of the vessels, 
especially in the hand, are not uncommon. In such instances 
the hands become bluish and icy cold, and we have a condition 
known as local asphyxia. Raynaud's disease may be con- 
founded with peripheral neuritis, ergotism, diabetes, and senile 
gangrene. It should, however, not be difficult to avoid such a 
mistake if we take into consideration the characteristic course 
of the disease and the absence of any of the astiological factors 
before mentioned. In the treatment favorable results have 
been repeatedly obtained by bathing the hands in warm water 
and the application of alcoholic menthol solution with a camel's- 

hair brush. 
26 



4 02 DISEASES OF THE SPINAL NERVES. 

LITERATURE. 
Weiss. Ueber sogen. symmetrische Gangran. Zeitschr. f. Heilk., 1882, iii, p.. 

233- 
Frantzel. Zeitschr. f. klin. Med., 1883, vi, 3, p. 277. 
Lutz. Bayr. arztl. Intell.-Bl., 1884, xxxi, 24. 
Schulz. Deutsch. Arch. f. klin. Med., 1884, xxxv, p. 183. 
Vulpian. Gaz. des hop., 1884, 9. 
Lauer. Ueber locale Asphyxie und symmetrische Gangran der Extremitaten. 

Inaug.-Diss., Strassburg, 1884. 
Pitres et Vaillard. Arch, de Phys., January, 1885, 3me ser., v, p. 103. 
Hochenegg. Ueber symmetrische Gangran und locale Asphyxie. Wiener med. 

Jahrb., 1885, 4, pp. 569-658. 
Shaw. Raynaud's Disease. New York Med. Journ., December 18, 1886. 
Powell. Brit. Med. Journ., January 30, 1886, p. 203. 
Goldschmidt. Gangrene symmetrique et sclerodermic Revue de med., Mai, 

1887, p. 404. 
Wigglesworth. Peripheral Neuritis in Raynaud's Disease. Brit. Med. Journ.,. 

January 8, 1887. 
Potain. Gaz. des hop., July 26, 1887, lx, 90. 
Fox, R. Hingston. Lancet, December, 1888, ii, 25. 
Tannahill. Glasgow Med. Journ., December, 1888, xxx, 6. 
Bramann. Falle von symmetrischer Gangran. Deutsche Med.-Ztg., 1889, 37, 

P- 432. 
Sturmdorf (New York). Symmetrical Gangrene. Med. Record, May, 1891,40. 
Scheiber. Wiener med. Wochenschr., 1892, 39-42. 
Kornfeld. Wiener med. Presse, 1892, 47, 48, 50, 51. 
Haig. Transactions of the Med. Society of London, 1892. 
Dehio. Deutsche Zeitschr. f. Nervenhk., 1893, iv. 
Germer. Raynaud'sche Krankheit. Inaug.-Diss., Berlin, 1893. 

Undoubtedly a close relation exists between Raynaud's dis- 
ease and scleroderma. In this latter very rare affection, which 
also depends upon trophic disturbances, the skin, after having 
presented cedematous swellings in the first stage, becomes later 
hard and immovable, so that it is impossible to pick up a fold 
of it between the fingers. The affected parts, more particularly 
the face, neck, and the upper portion of the chest, where fre- 
quently a diffuse increase in the pigment is noticeable, are im- 
peded in their movements, the play of the features is lost, the 
mouth can not be completely opened, the eyes can not be 
closed, and rotation of the head becomes impossible, etc. The 
patient feels a sensation of discomfort ; the coldness of the 
skin, which reminds one of that of a corpse, is most distressing, 
and a slight fall in the outside temperature is sufficient to bring 
about cyanosis. Quite gradually the atrophic, the terminal, 
stage comes on, in which the skin gets as thin as paper, remain- 



HEMIATROPHIA FACIALIS. 403 

ing, however, firmly fastened to the underlying tissues, so that 
it is still impossible to pick up a fold. With these changes is 
associated an atrophy of the muscles, which has to be regarded 
partly as a tropho-neurosis, partly as an atrophy due to inac- 
tivity, and the patient becomes helpless and unfit for work. 
After the disease has lasted for several years, if convalescence 
has not set in in the second stage, a general marasmus develops 
which leads to a fatal issue. An effectual treatment is not 
known. Warm baths, simple ointments, the constant current, 
internally tonics, iron, cod-liver oil, etc., may be tried, but we 
are not justified in placing any confidence in them. 

LITERATURE. 

Thibierge. Revue de med., 1890, 4. 

Hoffa. Munch, med. Wochenschr., 1892, xxxix, 35. 

Newmark. Amer. Journ. Med. Sciences, 1892, civ, 3. (Complication of Sclero- 
derma and Hemiatrophy of the Face.) 

Vandervelde. Journ. de med., de chir., et de pharm., 1893, li, 35, p. 561. (No 
pathological changes were found in the nervous system.) 

The next affection to which we shall call attention is as re- 
markable as it is rare. According to our present ideas, it has 
also to be ranked among the tropho-neuroses. We are refer- 
ring to a very gradually developing atrophy of the face (some- 
times ushered in by pain and paresthesias), which may appear 
on one or both sides, and generally embraces equally the skin, 
the subcutaneous tissue, the muscles, and the bones. The begin- 
ning is usually as follows : Whitish spots appear on the skin of 
the face, which sink in more and more and are accompanied by 
a diminution of the fatty tissues below ; gradually the atrophy 
increases in extent, and nothing escapes with the exception of 
the musculature, and this only occasionally and for a certain 
time. The affected side is sunken in, and the skin assumes a 
whitish-brown discoloration. The bones, especially the upper 
jaw, and with it the teeth, atrophy ; the latter fall out, as well 
as the hair, which often appears of a light color or distinctly 
gray. The bone atrophy is the more marked the younger the 
patient at the onset of the disease (Virchow). If the disease is 
confined to one side only — hemiatrophia facialis — the median 
line forms a sharply defined border and the diagnosis is very 
plain. If both sides are affected, as happened in Eulenburg's 
case after measles (Lehrb. der Nervenkrankh., 1878, ii, p. 620), 
it may be more difficult to recognize the affection. The grooves 



404 



DISEASES OF THE SPINAL NERVES. 



and furrows which are found in the face greatly disfigure it 
(Fig. 124). The corresponding half of the tongue becomes 
small, and often presents gap-like retractions such as we de- 
scribed in hemiatrophy of the tongue (Figs. 29 and 30). Among 
the muscles not only those supplied by the facial, but also those 




Fig. 124.— Hemiatrophia Facialis (personal observation). 

supplied by the trigeminus, are implicated. The extension of 
the atrophic process to the neighboring shoulder and even to 
the upper arm is not unusual. Sensibility is not altered in the 
affected region. 

Most of the few cases observed clinically have never come 
to autopsy. Of great interest, therefore, was the result of an 
examination which Mendel was enabled to relate before the 
Berlin Medical Society, on such a patient who had died of 
phthisis, and who had previously been examined by Romberg, 
and later on by Virchow (Deutsche Med.-Ztg., 1888, xxxiii, p. 
407). On examining the origin of the trigeminus, all the other 
roots were found to be normal ; only in the descending root 
could atrophic changes be recognized, a fact which would in- 
dicate that the trophic fibres must be contained therein. 



DISEASES OF THE MUSCLES. 



405 



Other observations (Ruhemann) also point to a very inti- 
mate connection between facial atrophy and the trigeminus ; 
however, more post-mortem observations confirmatory of those 
of Mendel are needed to clear up the pathology of the disease. 

Of the aetiology little that is positive is known. Age and 
sex seem to have little influence. The disease has been known 
to occur at all times of life and also to be congenital ; it has 
been observed in both sexes. According to Lewin, the fre- 
quency with which the two sexes are attacked is in the ratio of 
six males to nine females. Hereditary predisposition is cer- 
tainly not a conditio sine qua non, because there are cases — among 
others, the one I have reported myself — in which the patients 
belonged to quite healthy families. Sometimes it has happened 
that the atrophy was preceded by other nervous affections — 
trigeminal neuralgia, migraine, epilepsy, etc. — but this is by no 
means the rule. In a few cases the disease has followed in- 
juries about the face or of the cervical sympathetic (Seelig- 
miiller) ; more frequently no cause whatever could be demon- 
strated, and it was impossible to make any conjecture as to the 
aetiology. The outlook for recovery is absolutely bad, and 
therapeutics, so far as our knowledge goes, is powerless. 

LITERATURE. 

Eulenburg. Ueber progressive Gesichtsatrophie und Sclerodermic Zeitschr. f. 

klin. Med., 1882, v, 4. 
Wolff, J. Virchow's Archiv, 1883, xciv, 3, p. 393. ' 
Putzel. A Case of Progressive Facial Hemiatrophy. The Med. Rec, April 16, 

1887. 
Barwise. Lancet, December 31, 1887. 
Herz. Archiv f. Kinderheilkunde, 1887, viii, p. 241. 
Lowenfeld. Ueber einen Fall halbseitiger Atrophie der Gesichts- und Kaumus- 

culatur. Munch, med. Wochenschr., 1888, xxxv, 23. 
Mendel. Ueber Hemiatrophia facialis. Deutsche Med.-Ztg., 1888, 33. 
Lowenfeld. Munch, med. Wochenschr., 1888, 23, 24. 
Sachs, B. Progressive Facial Hemiatrophy with some Unusual Symptoms. 

Med. Record, March 15, 1890. 
Hoppe-Seyler. Deutsches Arch. f. klin. Med., 1889, xliv, 5, 6. 
Ephraim. Berliner klin. Wochenschr., 1889, 3, p. 55. 
Ruhemann. Deutsche med. Wochenschr., 1889, 3, 55. 
Fromhold-Treu. Ueber Hemiatrophia fac. prog. Inaug.-Dissert, Dorpat, 1893. 

APPENDIX. — DISEASES OF THE MUSCLES — PRIMARY MYOPATHIES. 

Diseases of the muscles, which consist in alterations in 
their volume (which is generally diminished, less frequently 



4 o6 diseases oe the spinal nerves. 

increased), and consequent disturbances of function, may occur 
under the most varied conditions. They may be produced by 
cerebral affections, as we have pointed out on page 232, where 
we spoke of the possibility of an affection of trophic centres in 
the cortex ; they may be the result of spinal diseases, as we 
shall later find out in discussing syringomyelia and progressive 
muscular atrophy, but they may also — and this is what inter- 
ests us here more especially — occur independently of any cen- 
tral disease as primary myopathies. 

Considered from an anatomical standpoint, this disturbance 
in the nutrition of the muscles, the dystrophia muscularis pro- 
gressiva of Erb, the myopathia progressiva primitiva of Char- 
cot, consists either in a diminution, a wasting of the muscular 
tissue, owing to which the volume of the part affected becomes 
smaller; or during the pathological process there may come 
about an increase in volume, which is either due to an actual 
increase and abnormal growth of the muscles — a genuine mus- 
cular hypertrophy — or to a growth of the interstitial fatty 
tissue, in which latter case we speak of a pseudo-hypertrophy 
of the muscles. Sometimes both conditions are found in one 
and the same individual, so that certain muscles appear atro- 
phied, while others, in consequence of the simultaneous de- 
velopment of fat, appear strikingly large and hypertrophied. 
The microscopical examination (Oppenheim and Siemerling, 
Miinzer, and others) shows, besides an increase of connective 
tissue, a moderate development of fat, and in the pseudo-hyper- 
trophic tissue a considerable increase in the interstitial con- 
nective tissue between the individual fibres, which latter have 
retained their transverse striation (Charcot, F. Schultze, 
Strumpell). The genuine hypertrophy which is seen in places 
must be regarded, according to Strumpell, as compensatory. 

About the aetiology of primary myopathies we know very 
little. It should, however, be mentioned that, according to all 
the observations made up to the present time, they belong en- 
tirely to early life, developing as they do before the twentieth 
year. Heredity plays frequently a role in the disease, since not 
uncommonly several cases occur in the same family. Dahn- 
hardt raises the question (Neurol. Centralblatt, 1890, 22) whether 
there might not occur a lesion of the spinal cord during fcetal 
life or during the act of birth ; if this should be shown to be 
true, the mother or, as the case may be, parturition will have 
to be regarded as an ^etiological factor. 



JUVENILE MUSCULAR ATROPHY. 



407 



Lesage has shown that they also may follow certain other 
diseases, as, for example, typhoid fever (cf. lit.). In such in- 
stances we have to deal with a secondary lipomatosis, develop- 
ing in circumscribed areas of the body, as the result of certain 
arterial changes. 

In our present state of knowledge we seem justified in as- 
suming that these myopathies occur regularly in certain groups 
of muscles, so that different " types " can be distinguished, and 
that on the whole the upper half of the body, particularly the 




Fig. 125. — So-called Juvenile Muscular Atrophy (Erb) (personal observation). 

upper extremities, are more often and more severely attacked 
by the pathological process than the lower parts, especially the 
legs. The latter, however, may also be affected, in which case 
the muscles supplied by the peroneus are especially apt to suf- 
fer (Sachs, The Peroneal Form of Leg Type of Progressive 
Muscular Atrophy, Brain, 1890). It is important always to 
observe whether the face remains intact or not, as in the for- 
mer case we are dealing with the hereditary muscular atrophy 



4o8 



DISEASES OE THE SPINAL NERVES. 



which Erb has described as the "juvenile form"; in the latter, 
with the form which Landouzy and Dejerine have described, 
and which has by them been called " myopathic atrophique 
progressive." 

The so-called "juvenile muscular atrophy " which develops 
in early youth, more often in boys than in girls, attacks by 





Fig. 126.— Juvenile Muscular Atrophy (Erb) (personal observation). 

preference the pectorales, the trapezius, the latissimus dorsi, 
the serratus magnus, the rhomboidei, the sacro-lumbalis, and 
the longissimus dorsi, while the majority of the muscles of the 
forearm, the sterno-cleido-mastoideus, the levator anguli scapu- 
lae, the coraco-brachialis, the teretes, the deltoid, the supra- 
spinatus and infraspinatus, remain, as a rule, intact. The small 
muscles of the hand, which in spinal atrophy become affected 
so early and in such a typical manner, are here not implicated 



JUVENILE MUSCULAR ATROPHY. 



409 



(Fig. 126). It is hardly necessary to enter into a description of 
the disturbances of function which necessarily must result from 
disease of so many muscles. If we remember how much im- 
paired are the movements of the arm, which can not be raised 
above the horizontal position, etc., we can understand the 
gravity of the child's affliction. If, as in the long course of 







Fig. 127.— Juvenile Muscular Atrophy (Erb) (personal observation). 

the disease not uncommonly happens, the process extends to 
the lower half of the body, the glutei, the peronei, the quadri- 
ceps, and tibialis anticus become implicated and the patients 
at first walk with an uncertain gait, then waddle in a char- 
acteristic manner, and finally lose the use of their legs. The 
implication of the muscles supplied by the bulbar nerves? 
which has been observed by Bernhardt (cf. lit.), of course has 
a very decisive influence upon the duration and course of the 
disease. 

Fibrillary twitchings in the affected muscles, as we see it so 



4io 



DISEASES OF THE SPINAL NERVES. 



commonly, we may say regularly, in the so-called progressive 
muscular atrophy (spinal), is here wanting with the same regu- 
larity. Neither can any changes in the electrical excitability 
be demonstrated, with the exception, of course, of a diminution 
due to the disappearance of a more or less large number of 
muscle fibres. The course of the disease is, as we said, emi- 
nently chronic. It may extend over a space of twenty or thirty 




Fig. 128. — Juvenile Muscular Atrophy (Erb) (personal observation). 

years, since bulbar symptoms occur but rarely, and we can 
only look for a fatal issue if the process involves the diaphragm 
and respiratory disturbances result. The diagnosis never pre- 
sents any difficulties. After careful examination, taking into 
account the distribution of the atrophy, the onset of the disease 
in early life, with the fact that more than one member of the 
family are affected, the long duration of the disease, the absence 
of fibrillary twitchings, we can not mistake the myopathic for 



PROGRESSIVE ATROPHIC MYOPATHY. 



411 



the spinal form. The treatment must consist chiefly in good 
care and nursing. All attempts to arrest the process by the 
application of electricity or the use of massage, baths, or in- 
ternal medicines, have proved to be of no avail. 

The facio-humero-scapular type of muscular atrophy of 
Landouzy and Dejerine, the " progressive atrophic myopathy," 
a form which had, however, already been described by Du- 
chenne under the name of " progressive muscular atrophy of 
infancy," may manifest itself before the appearance of any 
other symptoms by a markedly late development of the intel- 
ligence (Pillet, Revue de med., 1890, 5). The atrophy begins 




Fig. 129. — Progressive Atrophic Myopathy. Inability to close the eyes completely. 
(After Marie and Guinon.) 

in the muscles of the face, and our attention is attracted by the 
listless, sleepy expression of the face, the smooth forehead, the 
faulty movements of the mouth, the inability to whistle and 
to keep the lips together. At the same time there is a condi- 
tion of lagophthalmus, so that the patient, in spite of the great- 
est exertion, is unable to shut his eyes (cf. Fig. 129). Gradually 
the muscles of the upper extremities and the trunk become 
affected in almost the same distribution as in the juvenile paral- 
ysis. The course is the same in both these forms. Here also 
there are no fibrillary twitchings and no changes in the elec- 
trical excitability, and although the pseudo-hypertrophy of the 



4 I2 DISEASES OF THE SPINAL NERVES. 

muscles, which we shall presently describe, is not uncommon 
in the juvenile form and is here never present, there is no ques- 
tion but that the two affections are identical, and that only in 
some cases, from reasons not as yet understood, the interstitial 
connective tissue becomes early increased, while in others 
nothing else can be demonstrated but simple atrophy, with in- 
crease in the number of muscle nuclei and here and there the 
formation of vacuoles in the fibres. The diagnosis is so much 
facilitated by the " myopathic facies " — that is, the expression 
produced by the sinking in of the cheek, the somewhat depend- 
ent lower lip, and the inability to close the eyes — that the ex- 
perienced diagnostician is frequently able to recognize the 
disease at the first glance. Marie and Guinon have called 
attention to the possibility of confounding the disease with 
lepra ansesthetica, in the course of which also weakness of the 
facial muscles exists (cf. lit.). It is interesting to note in this 
connection that sometimes disturbances of function in the facial 
muscles may constitute a congenital defect which under cer- 
tain circumstances may be followed by an actual atrophy ; 
further, that in sisters or brothers of individuals who suffer 
from this myopathy which we have just described, a certain 
imperfection in the development of the facial muscles may be 
found, although the disease never breaks out in them. These 
are facts which Striimpell especially has pointed out, but the 
cause still remains wholly unexplained. About the treatment 
we need add nothing to what we have said with reference to 
the juvenile form. 

The third form of the muscular diseases now under consid- 
eration — the so-called pseudo-hypertrophy — is connected with 
an increase in the interstitial adipose tissue which, in spite of 
the atrophy of the muscle fibres, lead to an apparent increase 
in the volume of the affected parts. The disease was known 
and described by Griesinger in 1864, and again by Duchenne 
in 1868. It begins generally in the muscles of the trunk and 
attacks, in contradistinction to the two forms just described, 
by preference the lower parts of the body, the muscles of the 
back, loins, and thighs. Though for a long time the patient 
can use his arms and hands just as well as usual, the walk, 
owing to the affection of the erector muscles of the spine, 
becomes altered in the characteristic manner which we have 
described on page 363. The condition of the patient may re- 
main unchanged for years before the arms also take part in 



PSEUDO-HYPER TROPHIC MUSCULAR ATROPHY. 



413 



the process. When this happens it occurs in the same man- 
ner as in the juvenile form. The diagnosis is very much 
facilitated by the appearance of the patient. The enlarge- 
ment of the calf muscles, the thighs, and the glutei (which 
are sometimes colossal), give to him the 
appearance of a giant and suggest a su- 
pernatural strength (cf. Fig. 130) ; but 
the fact that these great masses feel 
spongy and soft, and that the electrical 
excitability is considerably decreased 
owing to the diminution in the number 
of the muscle fibres, readily explains why 
these sturdy-looking persons are feeble 
and without strength, and almost wholly 
deprived of the use of their limbs. 

In its onset the disease resembles 
closely the other forms. Here also only 
children become affected, more especial- 
ly those between the ages of four and 
nine. Again, the disease may occur in 
several members of the same family, so 
that we must undoubtedly assume a he- 
reditary predisposition ; and here also 
the fibrillary twitchings are not met 
with. Duration and treatment are the 
same as in the juvenile atrophy. 




Fig-. 130. — Pseudo-hyper- 
trophy of the Muscles 
of the Legs with At- 
rophy of the Muscles 
of the Back. (After Du- 

CHENNE.) 



Congenital atrophy of the muscles 
may be found in cases of malformation 
of the arms and hands. Fig. 131 repre- 
sents a boy aged thirteen in whom the 
forearms are absent ; some of the fingers 
are grown together and some deformed. 
A similar case has been reported by 

Wilkin (Lancet, page 1265, December 14, 1887), where there 
was atrophy of the biceps and the brachialis anticus. 

Absence of certain individual muscles is rarely observed. 
Erb has reported a case in which there was an almost entire 
absence of both trapezii (Neurolog. Centralbl., i, 1889). Among 
earlier instances the pectorales (Ziemssen), the biceps (McAl- 
lister), the deltoid, and gastrocnemius (Gruber), were wanting. 
These cases possess no clinical interest. 



414 



DISEASES OF THE SPINAL NERVES. 



The sensory disturbances which are peculiar to the mus- 
cles, but about the exact anatomical nature of which we know 
nothing, are called myalgias or muscular rheumatisms. ^Etio- 
logically, overexertion, strains (possibly rupture of certain mus- 
cle fibres, which may happen during gymnastic exercises or 
other violent bodily exertion), must be mentioned in this con- 
nection. Sometimes we are unable to find any such cause, 
and we have to attribute the trouble to the influence of cold. 




Fig. 131. — Absence of the Forearms. The fingers are partly grown together. Atrophy 
of the muscles of the upper arms (personal observation). 

There are persons who for years or tens of years suffer from 
myalgic pains which come and go and may disappear for cer- 
tain periods of time completely, and it is just possible that 
chronic intoxications — e. g., alcoholism, perhaps also circula- 
tory disturbances — have a predisposing influence. Among 
such myalgias, which may be very painful, even sufficiently 
so as to interfere with the occupation of the patient for a 
longer or shorter period of time, we have, for instance, the 
torticollis rheumatica, in which the muscles of the neck, the 



MYALGIAS. 



415 



myalgia lumbalis (lumbago), in which the muscles of the loins, 
the myalgia intercostalis, in which the intercostal muscles are 
attacked. The shoulder muscles may also be affected, and 
the myalgia in this region may become very obstinate without 
any implication of the brachial or cervical plexuses being de- 
monstrable. In the diagnosis we must think of the possibility 
of an implication of the nerves and endeavor to exclude neu- 
ralgia. We must further remember that central diseases may 
give rise to muscular pains. The inexperienced may mistake 
the lancinating pains of tabes for chronic muscular rheumatism, 
and thus obscure the correct diagnosis for years. It will hardly 
be difficult to avoid confounding muscular rheumatism with 
articular rheumatism if we take into consideration the gen- 
eral condition of the patient, the appearance of the joints, the 
temperature, pulse, etc., which in the former affection remain 
normal. 

In the treatment we should first of all endeavor to detect 
any underlying cause, and, if such exists, remove it. In re- 
cent cases, besides subcutaneous injections of morphine, sali- 
cylic acid may be tried internally ; yet we should not spend 
much time with it if we perceive no effect, but should rather 
prefer local applications — irritants to the skin, poultices, mus- 
tard plasters, liniments, also massage and electricity — especially 
if the affection remains localized. If this is not the case, but if 
the pains travel round the body and the course assumes a 
more chronic type, treatment by sweating, steam baths, also 
mud baths or baths of Pimis silvestris, the non-medicated hot 
springs (Gastein, Johannisbad, Teplitz) or the sulphur springs, 
among others Pistyan, in Hungary, will be recommended. As 
a last resort, we may advise the patient to go to a well-conducted 
hydrotherapeutic establishment (Grafenberg, Kaltenleutgeben, 
Nassau, etc.). 

LITERATURE. 

Landouzy et Dejerine. De la myopathic atrophique progressive. Revue de 

med., Fevrier-Mars, 1885. 
Marie et Guinon. Formes cliniques de la myopathic progressive primitive. Ibid., 

Octobre, 1885. 
Westphal. Ueber einige Falle von progressiver Muskelatrophie mit Betheili- 

gung der Gesichtsmuskeln. Charite-Annalen, 1885. 
Charcot. Revision nosographique des atrophies musculaires progressives. 

Progr. med., Mars 7, 1885. 
Mossdorf. Neurol. Centralbl., 1885, iv, 1. (Implication of the Facial Muscles 

in the Juvenile Muscular Atrophy.) 



41 6 DISEASES OF THE SPINAL NERVES. 

Krecke. Munch, med. Wochenschr., 1886, xxxiii, 14-16. (Implication of the 
Facial Muscles in Muscular Atrophy.) 

Ladame. Contribution a l'etude de la myopathie atrophique progressive. Revue 
de med., Octobre, 1886. 

Landouzy et Dejerine. Nouvelles recherches sur la myopathie atrophique pro- 
gressive, etc. Revue de med., Decembre, 1888. 

Lichtheim. Ueber hereditare progressive Muskelatrophie. Schweizer Corr.- 
Bl., 1888, xviii, 19, p. 603. 

Bernhardt. Ueber eine hereditare Form der progressiven spinalen, mit Bulbar- 
paralyse complicirten Muskelatrophie. Virchow's Archiv, 1888, Bd. 115, 2. 

Lesage. Note sur une forme de myopathie hypertrophique secondaire a la 
fievre typhoide. Revue de med., 1888, viii, 11, p. 903. 

Sachs. Progressive Muscular Dystrophies. Journal of Nerv. and Ment. Dis- 
eases, November, 1888, xiii, 11. 

Stern. Ein Fall von progressiver Muskelatrophie (juvenile Form, Erb), mit halb- 
seitiger Betheiligung des Gesichtes. Mittheil. aus d. med. Klinikin Konigs- 
berg. Leipzig, Vogel, 1888. 

Lichtheim. Ueber hereditare progressive Muskelatrophie. Centralbl. f. Ner- 
venheilk., 1888, xi, 20. 

Souza, Antonio Veiga de. Zwei Falle von juveniler Form der Muskelatrophie. 
Inaug.-Dissert., Kiel, 1888. 

Troisier et Guinon. Deux nouveaux cas de myopathie progressive primitive 
chez le pere et la fille. Revue de med., 1889, ix, 1. 

Remond. Une observation d'atrophie musculaire myelopathique a type scapulo- 
humeral. Progr. med., 1889, 2. 

Winkler en van der Weyde. Primaire myopathie (type facio-scapulo-humeral) 
gecombineerd med ophthelmoplegia progr. superior. Nederl. Weekbl, 
1889, i, 3. 

Scheuthauer. Histol. Untersuchung eines Falles von Pseudohypertrophic der 
Muskeln. Arch. f. Psych, u. Nervenkr., 1889, xx, 2. 

Herringham. Muscular Atrophy of the Peroneal Type affecting many Members 
of a Family. Brain, 1889, xi, p. 230. 

Pal. Ueber einen Fall von Muskelhypertrophie mit nervosen Symptomen. 
Wiener klin. Wochenschr., 1889, ii, 10. 

Auerbach. Zur Frage der wirklichen oder scheinbaren Muskelhypertrophie. 
Centralbl. fur die med. Wissensch., 1889, 45. 

Limbeck. Fall von completem Cucullarisdefect. Prager med. Wochenschr., 
1889, xiv, 36. 

Hitzig. Arch. f. Psych, u. Nervenkh., 1889, xxi, 2, p. 650. 

Stintzing. Deutsches Arch. f. klin. Med., 1889, 45, 3, 4. (Congenital and Ac- 
quired Defect of the Pectoral Muscles.) 

Gombault. Sur l'etat des nerfs peripheriques dans un cas de myopathie pro- 
gressive. Arch, de med. experim. et d'anat. path., 1889, 5. 

Duda. Fall von Pseudohypertrophic der Muskeln. Inaug.-Dissert., Berlin, 
1889. 

Muselier. Maladies generates chroniques et amyotrophiques. Gaz. med., 
1889, 20. 

Klaas van Roon. Over chronische en progressive atrophie van spieren. Akad. 
proofsch., Utrecht, 1889. 



PRIMARY MYOPATHIES. 



4*7 



Lenoir et Besangon. Myop. progr. primit. (type Landouzy). Revue de med., 

1890, 4. 
Annequin. Arch, de med. et de pharm. mil., 1890, xv, 4. (Atrophy of the 

Rhomboid Muscles.) 
Bruns et Kredel. Fortschr. d. Med., 1890, 1. (Congenital Defect of the 

Pectoral Muscles.) 
Bielschowsky. Neurol. Centralbl., 1890, 1. 
Spillmann et Haushalter. Revue de med., 1890, 6. 
Rovighi e Levi. Contribuzione alio studio della distrofia muscolare progressiva. 

Reggio Emilia, 1891. 
Guttmann, P. Deutsche med. Wochenschr., 1891, 34. 
Krauss, William C. Muscular Atrophies. A Clinico-Pathological Study. The 

Buffalo Medical and Surgical Journal, April, 1891. 
Israel, A. Ueber Dystrophia musculorum progressiva. Inaug.-Dissert., Frei- 
burg, i. B., 1891. 
Erb, W. Dystrophia muscularis progressiva. Volkmann's Samml. klin. Vortr., 

Neue Folge, November, 1892, 2. 
Muenzer. Zur Lehre von der Dystrophia muse, progressiva. Zeitschr. f. klin. 

Med., 1893, xxii, 6. 
Senator. Ueber acute Polymyositis und Neuromyositis. Deutsche med. 

Wochenschr., 1893, 39. 
Higier (Warsaw). Ueber prim are und secundare Amyotrophien organischer 

und dynamischer Natur. Ibid., 1893, 38, 39. 
Striimpell. Deutsche Zeitschr. f. Nervenhk., 1893, p. 471. 



PART III. 

DISEASES OF THE SUBSTANCE OF THE SPINAL 

CORD. 

Diseases confined to the substance of the spinal cord are 
rarer than those of the brain substance. The cause of this may 
lie in the fact that not only are the vessels of the spinal cord 
actually less frequently the seat of disease than those of the 
brain, but also that when they become diseased the conse- 
quences entailed are generally not of so grave a nature as 
those resulting from lesions of the cerebral vessels. 

As in cerebral diseases, here also two questions must ever 
be kept in view by the physician : (i) Where is the spinal lesion 
situated ? (2) What is its nature ? As we shall see later, it is 
especially the second which is of importance for the prognosis 
and choice of treatment. Both, however, are of equal weight 
for the proper recognition and conception of a given case. As 
in the study of the brain lesions, the topical and pathological 
diagnosis should here no less go hand in hand. 

I. Consideration of Spinal Diseases with Eeference to 
their Seat— Topical Diagnosis. 

As a thorough acquaintance with the anatomy of the parts 
is of the highest importance in making a topical diagnosis, 
some remarks on these points may in this place not be unwar- 
ranted. 

Without being separated by any sharp line of demarcation from 
the medulla oblongata, the spinal cord extends from the upper mar- 
gin of the arch of the atlas to the first lumbar vertebra, where it 
ends in the conus meduliaris. From this point it is seen as a long 
filiform continuation — the filum terminale. The cauda equina con- 
sists of the longitudinal nerve bundles which accompany the filum 
terminale, and corresponds to the lumbar and sacral part of the 
vertebral column. As it is apparent that the different pairs of nerves 
418 



ANATOMICAL RELATIONS. 



419 



do not leave the spinal cord at the level of 
the vertebrae after which they are named, 
but that they must necessarily do so higher 
up, it is important to know to what nerves 
certain parts of the vertebral column cor- 
respond. Thus we must remember that the 
first three cervical vertebrae correspond to 
the origin of the third, fourth and fifth cer- 
vical nerves, and that the seventh cervical 
vertebra corresponds to the first dorsal 
nerve. The spinous process of the fifth 
dorsal vertebra corresponds to the origin 
of the seventh, that of the tenth to the 
twelfth pair of dorsal nerves. Opposite 
the eleventh dorsal vertebra originates the 
first, between the eleventh and twelfth the 
second, opposite the twelfth the third and 
fourth lumbar nerves. Between the twelfth 
dorsal and first lumbar vertebra the fifth 
lumbar and first sacral nerves take their 
origin, the other sacral nerves opposite the 
first lumbar vertebra. The cervical en- 
largement corresponds, therefore, to the 
spinous processes of the cervical vertebrae, 
the lumbar enlargement to the spinous pro- 
cesses of the last dorsal vertebrae. All 
these relations, and, moreover, the fact 
that the spinous processes, which alone can 
be our guides, are not always on the same 
level as their corresponding vertebrae, are 
demonstrated in Fig. 132. 

The relation between the white matter 
and the gray which it incloses becomes ap- 
parent in a transverse section of the spinal 
cord. Here we see also that an anterior 
and a posterior fissure divide the spinal 
cord into two halves. These fissures, how- 
ever, do not meet, but are separated from 
each other by the so-called "commissures " 
which connect the two halves of the cord. 
The anterior part of the gray matter, the 
so-called " anterior horn," does not present 
the same diameter and form throughout, 
and in the cervical and lumbar enlarge- 
ment is larger than in the dorsal part of 



C.l 



#14" 



m 






20 



m 



m 



m 



F:g 132.— The Relations of 
the Origin of the Nerves 
to the Bodies of the Ver- 
tebrae and the Spinous 
Processes. (After Gowers.) 



420 



DISEASES OF SUBSTANCE OF SPINAL CORD. 




the cord (cf. Fig. 134). From this anterior horn proceed the ante- 
rior nerve roots and pass through the white matter which lies exter- 
nally. The posterior horn is much smaller and extends almost to 

the entrance of the posterior roots, 
which reach it after passing through 
the external part of the posterior 
columns (" root zone" of Char- 
cot). The arrangement of the 
white substance and its subdivi- 
sion into columns and tracts is 
determined (1) by the existence 
of the above-mentioned fissures, 
(2) by the entrance of the nerve 
roots, (3) by the shape of the gray 
matter. We distinguish roughly 
an " antero-lateral column " and 
a posterior column on each side. 
The former contain (a) the crossed 
lateral or pyramidal tracts, (b) the 
direct cerebellar tracts, (c) the an- 
terior direct pyramidal tracts, also 
called columns of Tiirck or un- 
crossed anterior columns. The posterior columns consist of the col- 
umns of Goll (at the inner side) and the columns of Burdach, which 
latter have also received the name " root zone " (cf. Fig. 133). 

Physiologically, the spinal cord is primarily important as a great 
conducting system, and next as the seat of numerous centres. The 
motor impulses originate in the brain, and travel down along the 
antero-lateral column chiefly in the crossed pyramidal tract of the 



Fig. 133.— Scheme of the Conducting 
Paths in the Spinal Cord at the 
Level of the Fifth Dorsal Nerve. 
(After Flechsig.) vzv, anterior, hw, 
posterior root. «, direct, g, crossed py- 
ramidal tracts, d, anterior column ground 
bundle, c, Goll's column, d, Burdach's 
columns, e and _/, mixed lateral paths. 
/i, direct cerebellar tracts. 






Fig. 134. — Cross-section through the Spinal Cord at Different Levels, a, level 
of the second, b, level of the seventh cervical vertebra, c, level of the second, d, level 
of the third lumbar vertebra. (After Quain.) 

opposite side, the decussation, as has been repeatedly pointed out, 
taking place for the most part in the medulla oblongata. Through 
the large ganglionic cells of the anterior horns these crossed pyrami- 
dal tracts are continued into the anterior nerve roots and leave 
as such the spinal cord. The sensory impressions are transmitted 
through the posterior roots, hence (some passing through the pos- 



THE REFLEXES. 



421 



tero-lateral columns) they reach the posterior horns and at once cross 
over to the opposite side of the spinal cord. The further course of 
the sensory fibres as they pass to the brain is not clearly understood; 
especially imperfect is our knowledge with regard to those for the 
different qualities of sensation — e. g., the sense of touch. It seems, 
however, that the central gray substance must be looked upon as 
the path for impressions of pain (cf. the investigations of Edinger 
about the continuation of the posterior spinal roots up to the brain, 
Anatom. Anzeiger, 1889, iv, 4). 

We know that reflexes originate by the stimulation of a sensory 
nerve. By this an impulse is conducted to a centre, and hence is 
transferred to a motor nerve — reflex arc (Fig. 135). Among such 




Fig. 135. — Reflex Arc. Af, motor path. 6", sensory path, m, motor (anterior), s, sensory 

^ posterior; nerve root. 



reflex movements we distinguish (1) skin reflexes, caused by irrita- 
tion of the skin, (2) tendon reflexes, which are produced by tapping 
on a tendon. To the former belong the plantar reflex, the centre for 
which is situated in the lower part of the lumbar enlargement, the 
gluteal, the anal reflex (Rossolimo, Neurol. Centralbl., 1891, 9), the 
cremasteric, and the abdominal reflexes, which are obtained by irri- 
tating the skin of the buttocks, the anus, the inside of the thigh, and 
the abdomen respectively. If we find these present in a patient we 
may assume the centres, which are situated in the lumbar and the 
dorsal cord respectively, to be intact. 

One of the diagnostically most important signs is the condition 
of the so-called patellar reflex. When the tendon of the quadriceps 



422 DISEASES OF SUBSTANCE OF SPINAL CORD. 

femoris is tapped, a reflex contraction of this muscle ensues by 
which the leg is jerked forward with more or less vigor. This is 
found in most healthy persons. It has been called by Erb "patellar 
tendon reflex"; by Westphal, who doubted its reflex nature, "knee 
phenomenon"; by Gowers, "knee jerk." 

To a certain extent the mode of tapping this tendon and the 
position of the patient are matters of indifference. The only points 
to remember are these : The lower leg should be held perfectly loose, 
and no superfluous clothing should prevent the proper striking of 
the tendon. The simplest way is to place the patient on the edge of 
a table, remove all clothing from his legs, then, while conversing 
with him about indifferent matters so as to distract his attention 
from what is going on, to observe the effect of the percussion of the 
patellar tendon. The exact determination of the strength of the 
reflex by means of the reflexograph (Bechterew, Neurol. Centralbl., 
1892, 2) can be dispensed with in every-day practice. 

If we find the reflex present, we may at once conclude that the 
spinal cord at a certain place — that is, from the second to the fourth 
lumbar or first sacral nerves, according to Westphal — is intact. 

If, on the other hand, the reflex is not obtained on the first and 
after repeated examinations, the patient ought to be directed to in- 
terlock his bent fingers and pull strongly (Jendrassik), and only if 
the knee jerk does not occur after repeated trials in the way de- 
scribed, should we assume its absence (Jendrassik, Neurol. Central- 
blatt, 1885, 18). It has for some time been Jendrassik's experience 
that the tendon reflexes, more particularly the patellar reflex, is 
much enforced if the other muscles of the body are put into strong 
action (Deutsch. Arch. f. klin. Med., xxxiii). This method of Jen- 
drassik is an excellent and indispensable means in doubtful cases for 
establishing the presence or absence of the knee jerk. Sternberg has 
recently investigated various conditions under which the tendon re- 
flexes meet with inhibiting, diminishing, or increasing influences in 
the spinal cord (Die Sehnenreflexe und ihre Bedeutung fur die Pa- 
thologie des Nervensystems, Leipzig und Wien, Deutike, 1893). 

Besides the patellar reflex, the Achilles tendon reflex, and the 
ankle clonus (the foot phenomenon of Westphal) must be mentioned. 
The latter consists of a succession of clonic contractions of the 
tendo Achillis which occur on a sharp dorsal flexion of the foot. To 
the violent shaking movements of the whole leg, which occasionally 
occur under these conditions, the very inappropriate name of spinal 
epilepsy has been given. 

If the reflex excitability is much increased, a simple tapping on 
the front of the lower leg is sufficient to produce a contraction of the 
calf muscles. This is what the English writers call the " front tap." 



LOCALIZATION OF SPINAL CORD LESIONS. 



423 



Whether all these so-called tendon reflexes are really of reflex 
nature, or whether they are not rather phenomena due to a direct 
stimulation of the muscles (Westphal), is still an unsettled ques- 
tion. 

The same uncertainty exists about a symptom which has by West- 
phal been termed "paradoxical contraction," and which consists in a 
muscle remaining in tetanic contraction for quite a time after it has 
been passively shortened. For instance, if we flex the foot of a 
patient lying in bed, the tibialis anticus may under certain conditions 
remain for some time in a state of contraction ; its tendon becomes 
prominent, and only gradually relaxes and allows the foot to return 
to its normal position of rest. Only rarely has this phenomenon 
been observed in other muscles. 

Further, reflex centres are found in the lumbar region of the 
spinal cord for the emptying of the bladder and rectum, for the erec- 
tion of the penis and the ejaculation of the semen — reflexes which are 
concerned with the sexual functions. According to the researches of 
Sarbo (Arch. f. Psych., 1893, xxv, 2) the centre is situated between 
the levels of the first and fourth sacral nerves. 

LITERATURE. 

Lombard. Die Variationen des normalen Kniestosses und deren Verhaltniss 
zur Thatigkeit des Centralnervensystems. Arch. f. Anat. u. Physiol., 1889, 
Supplementband, p. 292. 

Kupferberg. Beitrag zur Kenntniss der Hautreflexe bei Nervengesunden. 
Inaug.-Dissert., Freiburg, 1889. 

Benedict. Einige qualitative Varietaten des Kniephanomens. Neurol. Centralbl., 

1889, 19. 

Erben. Neue Beitrage zur Kenntniss der Reflexe. Wiener med. Wochenschr., 

1 890, xl, 2 1 et seq. 

Dinkier. Localisation und klinisches Verhalten der Bauchreflexe. Deutsche 

Zeitschr. f. Nervenhk,, 1892, ii, 4. 
Eichhorst. Paradoxer Patellarsehnenreflex. Centralbl. f. klin. Med., 1892, 31. 
Geigel. Die klinische Priifung der Hautreflexe. Deutsche med. Wochenschr., 

1892, 8. 
Sternberg. Die Sehnenreflexe und ihre Bedeutung fur die Pathologie des 

Nervensystems. Leipzig und Wien, Deuticke, 1893. 

With regard to the localization of the spinal cord lesion, 
two questions arise: (1) Which portion of the cord is dis- 
eased ? Is it the cervical, dorsal, or lumbar? (2) Which part 
of the cross section of the cord ? Is it the gray or the white 
matter, or both ? The first question can be answered without 
difficulty in cases where the vertebral column is diseased ; we 
only need to examine the latter by pressing upon the vertebras 
or by applying a hot sponge, etc., over them. Those spots at 



424 DISEASES OF SUBSTANCE OF SPINAL CORD. 

which tenderness is elicited by the application are the seat of 
the disease. The occurrence of spontaneous pain is rarer in 
diseases of the cord. It should, above all, be remembered that 
lesions of the spinal cord, as such, wherever they may be, 
almost never produce pain in the back, but that this is in a 
majority of cases due to trouble in the muscles or their nerves. 
It is a characteristic feature of these pains that they become 
especially marked after prolonged standing and stooping, and 
that the}' are very bad on rising in the morning. They may 
occur sometimes after a quick movement, in which case some 
muscle bundles have been overstretched or even torn. Pains 
in the back which persist for months and years unaffected by 
any therapeutic measures justify a suspicion of the existence 
of an aortic aneurism which may be pressing against the verte- 
bral column or of enlarged carcinomatous abdominal glands 
(Johnson, British Medical Journal, February 12, 1881). In dis- 
ease of the vertebral column, especially if it be cancerous, pain 
in the back is a prominent symptom, as we have said. 

But, leaving out the tenderness on pressure, there are other 
symptoms which may help us to decide what segment of the 
cord is diseased in a given case. 

Diseases of the cervical cord generally produce symptoms 
of motor or sensory irritation or of paralysis in the upper 
extremities, pains, paresthesias, feelings of weakness, jerkings, 
and the like in arms, hands, and fingers, to which may be 
added also trophic disturbances. Muscular atrophies and loss 
of reflexes in the upper extremities are often observed. The 
lower extremities, however, remain intact, and the patellar re- 
flex is present and sometimes increased. Repeatedly a very 
decided slowing of the pulse (as low as thirty-two beats to the 
minute in a case of Lebrun's, Bull, de l'Acad. de med. de Bel- 
gique, 1, 1887, 1) has been met with in lesions of the cervical 
cord, and has been attributed to a chronic state of irritation of 
the vagus due to compression or some similar influence. 

Affections of the dorsal cord are mostly accompanied by 
sensory disturbances, paraesthesias in the back, intercostal neu- 
ralgias, aching, boring pains, which sometimes radiate into the 
lower extremities. Anaesthesias, though they are not the rule, 
may be found. If a distinctly circumscribed zone of anaes- 
thesia is made out, it corresponds exactly to the place where 
the lesion in the spinal cord is situated (cf. what will be said 
about lesions of one half the cord on page 456). 



LESIONS OF THE GRA Y MA TTER. 



425 



Lesions of the lumbar cord entail symptoms in the lower 
extremities, giving rise to weakness and paralysis, sometimes 
also jerkings and stiffness; furthermore, to pains, numbness, 
anaesthesias of the legs and feet. The reflexes are lost and 
vesical and rectal symptoms are present, the former consisting 
of retention or dribbling of the urine, pains, strangury, etc. Of 
course, the symptoms may greatly vary according as the whole 
transverse section or only some or even one system of fibres 
alone is affected in the given level of the cord. Fracture of 
the first lumbar vertebra causes a lesion of the conus ter- 
minalis ; a lesion at the level of the second lumbar vertebra 
and below it gives rise to affections of the cauda equina; the 
clinical symptoms of these conditions have been ably described 
by Valentine, who worked under Lichtheim ; besides the 
symptoms above referred to, he has called attention to the 
atrophy of certain muscle groups (the glutei, flexors of the 
thigh, muscles of the lower leg and foot) and the reaction of 
degeneration occurring in them. 

An answer to the second question demands a thorough ac- 
quaintance with the symptoms produced by lesions of the dif- 
ferent portions of the cross section. These we will therefore 
now consider. 

I. LESIONS OF THE GRAY MATTER — " POLIOMYELITIS." 

In giving the name poliomyelitis (7ro\to?, gray) to all spinal 
affections confined to the gray matter, we must at once insist 
that these lesions are almost entirely limited to the anterior 
portion of the gray matter, the anterior horns, and more espe- 
cially to the large ganglionic cells in them. Other portions 
have only rarely been found affected, and then only in connec- 
tion with the just-mentioned lesion. The diseases of the gray 
substance proper which have come under observation were 
confined to the groups of ganglionic cells of which we have 
just spoken. Clinically, there are two such diseases to be dis- 
tinguished, namely, poliomyelitis anterior acuta, or spinal pa- 
ralysis of children (infantile spinal paralysis), and progressive 
muscular atrophy. 



CHAPTER I. 

POLIOMYELITIS ANTERIOR ACUTA INFANTILE SPINAL PARALYSIS. 

Infantile paralysis, first accurately described by Jacob von 
Heine in 1840, is one of the best-known diseases of the spinal 
cord, both as regards its anatomical seat and its clinical course. 
As has been demonstrated beyond doubt by Charcot, Prevost, 
and Joffroy, it is an acute inflammation of the anterior horns, 
or rather, as is usually the case, of one of them. This leads to 
an atrophy and sclerosis, so that a dense tissue remains, con- 
taining- the dilated vessels and small remains of ganglionic cells, 
which are not rarely found to be calcified (Friedlander, cf. 
Fig. 136). The seat of the process is usually either in the 




Fig. 136.— Transverse Section from the Cervical Portion of the Spinal Cord. 
Atrophy and sclerosis of the right anterior horn. (After Charcot. ) 



cervical or the lumbar enlargement. In the former case the 
paralysis affects the upper, in the latter the lower, extremity. 
The secondary degeneration, which ensues as a consequence of 
the atrophy of the ganglionic cells, extends to the anterior 
426 



INFANTILE SPINAL PARALYSIS. 



427 



nerve roots, the motor nerves, and the muscles supplied by 
them. It is a genuine degenerative atrophy, just as much as 
the one described as coming on after peripheral paralyses. 

Symptoms. — The clinical picture of the disease is very 
characteristic. The onset bears a striking resemblance to that 
of cerebral infantile paralysis, described on page 271. In the 
midst of perfect health the child is suddenly seized with head- 
ache, vague pains in the limbs, and fever, the temperature 
reaching 104 F. or even more ; he becomes stupid and som- 
nolent, and soon, while complete unconsciousness is developed, 
general convulsions set in, which last usually from one to three 
days and then disappear. The patient's condition becomes 
better, consciousness is fully regained, he becomes bright and 
talkative, and the relatives think that the malady has already 







Fig. 13:.— Spinal Infantile Paralysis (personal observation). 

spent itself, when unfortunately a more careful examination re- 
veals that the movements of the child are impaired, that one, 
more rarely both, upper or lower extremities are paralyzed. 
The paralysis, which usually affects one arm (Fig. 137) or one 
leg, has developed rapidly and reached a considerable extent, 



428 DISEASES OF SUBSTANCE OF SPINAL CORD. 

which, however, is rarely maintained. On the contrary, as 
a rule, it partially recedes and confines itself to certain mus- 
cles, which then remain permanently paralyzed. According 
to Beevor (cf. lit.), the affection sometimes embraces groups 
of muscles corresponding to those which Ferrier in his ex- 
periments on monkeys saw contract after stimulation of the 
different cervical nerve roots. In the majority of cases the 
paralysis takes in one leg. The paralyzed muscles rapidly 
atrophy, and the electrical excitability undergoes quantitative 
as well as qualitative changes — reaction of degeneration. The 
whole extremity is stunted in its growth, and even the bones 
may be found several centimetres shorter than those of the 
other leg. The appearance of such an extremity, in which at 
first all passive motions are possible, is quite characteristic. 
The skin is pale, cyanotic, and feels cold, but retains its sensi- 
bility completely. Skin and tendon reflexes are lost, but there 
are no vesical symptoms. Later on secondary contractures 
develop, among which the so-called " paralytic clubfoot " is the 
best known. In consequence of the paralysis of the peroneal 
muscles, their antagonists, the calf muscles, become perma- 
nently contracted and cause the point of the foot to hang 
down. In the arms analogous conditions may be found, the 
non-paralyzed antagonists always assisting in the production 
of the contractures. 

Roughly speaking, this is the course in most cases, only 
occasionally the initial fever may be slight enough to be over- 
looked and the paralysis develop without the child ever having 
taken to his bed. In rare cases the convulsions, instead of last- 
ing for days, continue for weeks. In others, again, several 
months may pass before the onset of the actual paralysis; but 
all these are the exceptions, which need not confuse us in mak- 
ing a diagnosis. The further general development (with the 
exception of that of the paralyzed extremity) is perfectly nor- 
mal, and neither, as happens in the cerebral infantile paralysis, 
does the mind become in any way impaired nor do the initial 
convulsions ever recur. The child grows up in good health, 
but always remains, especially if one leg is affected, a cripple. 
If, as often happens, contractures or a spontaneous paralytic lux- 
ation of the hip develop, the patient has for years to be under 
the care of the surgeon, and needs braces and the like. If an 
arm is affected, the capability of the patient for making his liv- 
ing is naturally considerably and permanently interfered with. 



INFANTILE SPINAL PARALYSIS. 



429 



Diagnosis. — It is not difficult to avoid mistaking the disease 
for any other if we bear in mind the characteristic onset, the 
localization, the behavior of the paralysis itself, the flaccid con- 
dition of the muscles, the absence of the reflexes, and the cold 
and cyanotic skin. Where we find a hemiplegia — i. e., where 
the arm and leg of the same side are paralyzed — we should in 
children always first think of infantile spastic hemiplegia (page 
271), as it is one of the rarest exceptions for the spinal paraly- 
sis to take on this distribution. Confusion with the syphilitic 
pseudo-paralysis, also known under the name of Parrot's dis- 
ease, is avoided by remembering the fact that in this disease 
the paralysis makes its appearance immediately, or at least with- 
in a few days, after birth (Dreyfouss, Revue de med., aout 1885, 
v), while Heine's paralysis of children does not occur at such 
an early age. 

Prognosis. — The prognosis, as soon as the initial acute 
symptoms have passed, is, so far as life is concerned, absolutely 
favorable ; so far as the recovery of function in the affected ex- 
tremity is concerned, equally unfavorable. Any notable im- 
provement is very rare, complete cure out of the question. 
These points should be carefully considered before inducing a 
poor and struggling father to let his child undergo year after 
year an expensive and useless course of treatment. 

^Etiology. — Of the aetiology of the disease we know noth- 
ing. It is doubtful whether cold is ever a causative factor. It 
is possible that infectious influences, the action of certain micro- 
organisms, will at some time be proved to be the cause of the 
disease. For the present, however, this is nothing more than 
a hypothesis which has not gained any firmer ground from the 
report of Cordier of an epidemic of the disease (Lyon med., 
1888, 1, 2). In a small village thirteen children were inside of 
two months taken ill with anterior poliomyelitis and four died. 
According to Cordier, the appearance of the disease in sum- 
mer, the sudden onset, the similarity in course, speak for an 
infectious origin, the infection, as he supposes, taking place 
through the air passages. 

Treatment. — Little more is known about the treatment than 
about the aetiology. All measures to cure or even merely to 
improve this rapidly developed paralysis are more or less use- 
less. Electrical treatment with the faradic or galvanic current, 
systematic massage, gymnastic exercises, together with rubbing 
with all sorts of salves — all these have been tried without any 



430 DISEASES OF SUBSTANCE OF SPINAL CORD. 

noteworthy success. In a few cases I have seen the method- 
ical use of heat, in the form of hot sand baths, warm packs, etc., 
bring about at least a perceptible improvement ; but even here 
this was out of proportion to the care and trouble which had 
been taken. Certain it is that the influence of the different 
baths has been greatly overrated, whether it be the brine 
baths of Kreuznach, Reichenhall, Kolberg, or the chalybeate 
springs of Pyrmont, Flinsberg, Schwalbach, or the sodium 
waters of Rehme, Soden, or, finally, the non-medicated hot 
springs of Gastein, Johannisbad, and many others, each of 
which has its advocates. The most appropriate appear to be 
those last mentioned, but in most cases we shall even then find 
that while perhaps the child's general condition is improved 
and it becomes strong owing to the good hygiene and fresh 
air, the paralysis, for the sake of which all has been undertaken, 
remains absolutely unchanged and presents no improvement. 

In view of these unsatisfactory results, the interesting but 
still scanty communications, according to which the growth 
of bone can artificially be increased, deserve our deep interest. 
In 1887 Helferich proposed to tie round the affected (paralyzed, 
atrophic) limb elastic rubber tubing so as to produce an arti- 
ficial engorgement, and through this a more active nutrition 
of all the tissues, including the bones. Schiiller also has re- 
ported before the Berlin Medical Society, November 28, 1888 
(Deutsch. Med.-Ztg., 1888, 99, page 1182), several cases which 
were thus treated and which showed decided improvement. 
To judge from his communication, this treatment undoubtedly 
should be tried in all suitable cases. It is, however, a proced- 
ure which, as well as the orthopaedic treatment so important 
for the prevention of deformities, should not be undertaken 
without consulting a surgeon. 

LITERATURE. 

Charcot. Lectures on the Diseases of the Nervous System, delivered at La 

Salpetriere. Translated by George Sigerson. New Sydenham Society, 

London, 1877. 
Friedlander, C. Ueber Verkalkung der Ganglienzellen. Virchovv's Archiv, 

1882, 88, 1. 
Rockvvitz. Deutsche Zeitschr. f. Chir., 1883, xix, 2, 3. 
Sahli. Deutsches Archiv f. klin. Med., 1883, xxxiii, 3, 4. 
Lorenz. Ueber die Entstehung der Gelenkscontracturen nach spinaler Kinder- 

lahmung. Wiener med. Wochenschr., 1887, 27-31. 
Karewski. Die der spinalen Kinderlahmung folgenden Gelenkscontracturen 



CHRONIC ANTERIOR POIIOMYEIITIS. 43! 

unci die paralytische Luxation der Hiifte. Archiv f. klin. Chir., 1888, 37, 2, 
p. 346. 

Rieder, Hermann. Poliomyelitis ant. acuta. Munch, med. Wochenschr., 1889, 
xxx vi, 2. 

Eulenburg. Subacute amyotrophische Spinallahmung mit paralytischer Schulter- 
gelenkerschlaffung, Behandlung durch Arthrodese. Berliner klin. Wo- 
chenschr., 1890, 4, 5, 38. 

Rosenberg. Die Differentialdiagnose der Poliomyelitis anterior acuta und 
chronica adultorum und der Neuritis multiplex. Inaug.-Dissert., Heidel- 
berg, 1890. 

Goldscheider. Ueber Poliomyelitis anterior. Zeitschr. fiir klin. Med., 1893, 
xxiii, Heft 5, 6. 

Siemerling. Acuter Befund bei spinaler Kinderlahmung. Deutsche Med.-Ztg., 
1891, 96. 

Kohnstamm. Schnittserien-Untersuchung eines Falles von spinaler Kinder- 
lahmung. Ibid., p. 556. 

Marie, P. Lecons sur les maladies de la moelle. Paris, Masson, 1892. (This 
work should be consulted for each chapter of this portion.) 

Though the lesions of the gray anterior horns when occur- 
ring in children are well understood, both in their anatomical 
and their clinical aspect, yet when the same process takes place 
in adults our knowledge becomes very limited. Here the ma- 
terial at our disposal is still so small that only in rare excep- 
tions can we say definitely whether we are dealing really with 
an anterior poliomyelitis and not rather with a peripheral dis- 
ease, a multiple neuritis. Clinically, the differential diagnosis 
between the two can only be made in the initial stage, as the 
peripheral disease is accompanied with pains and sensory dis- 
turbances which are absent in the central affection. 

A patient is taken ill with grave general disturbances — 
fever, somnolence, convulsions, delirium, etc. — and within a 
short time, perhaps in one or two weeks, a widespread paralysis 
in all four extremities is developed. The paralyzed muscles 
become flaccid and atrophy, the tendon reflexes disappear ; 
sensation, however, as well as bladder and sexual functions, 
present no abnormity. With a history like this we must think 
of a lesion of the anterior.gray horns. This idea becomes more 
than a conjecture if on examination the affected muscles are 
found to be such as are supplied from ganglionic cells, which 
most probably lie in close proximity to one another in the 
spinal cord. In such cases, as Remak has shown so beautifully, 
certain types of paralysis are observed — the forearm type 
(paralysis of all the extensors without the supinator longus) 
and the upper-arm type (paralysis of the biceps brachialis 



432 DISEASES OF SUBSTANCE OF SPINAL CORD. 

anticus, deltoid, and the supinator longus) — but unfortunately 
such instances are rare, and therefore even quite an experi- 
enced physician may feel uncertain about the diagnosis. 

The difficulty becomes greater if the paresis or paralysis is 
not extensive and does not develop rapidly, but slowly and by 
fits and starts. In these cases not rarely a temporary improve- 
ment may be noted and arouse hopes of complete recovery, 
unfortunately never justified. These are the instances in which 
we find not complete but partial reaction of degeneration in 
the paralyzed muscles — intermediate form of chronic anterior 
poliomyelitis (Erb). It goes without saying that we must have 
the other symptoms, especially the loss of reflexes, even to jus- 
tify a conjectural diagnosis. Moreover, it is necessary that there 
should be absolutely no sensory changes, and that bladder and 
sexual functions should be normal. Of the points of difference 
between anterior poliomyelitis and tabes we shall speak later. 

We can hardly expect much from any treatment. Elec- 
tricity, however, should be tried, if for no other reason than 
that something is done. Duckworth recommended, besides, 
belladonna, iron, quinine, and cod-liver oil, and claimed to have 
cured cases with these remedies. 

With reference to the setiology, nothing certain is known. 
Whether traumatism can ever cause anterior poliomyelitis re- 
mains doubtful, notwithstanding the report of Gibbons (Med. 
Times and Gazette, September 5, 1885). He had among his 
patients a boy nine years of age who after a fall on his knees 
developed the symptoms of an anterior poliomyelitis (and re- 
covered completely !). In cases of chronic anterior poliomye- 
litis which came to autopsy, sometimes atrophy in the gangli- 
onic cells of the anterior horns through the whole length of 
the cord, as well as atrophy of the anterior roots, was observed, 
while the peripheral nerves remained intact (Oppenheim). 

LITERATURE. 

Erb. Ueber das Vorkommen der chron.-atroph. Spinalahmung beim Kinde. 

Neurol. Centralbl., 1883, ii, 8. 
Bernhardt. Virchow's Archtv, 1883, Bd. 92, p. 369. 
Duckworth. Clinical Lecture on Subacute Anterior Spinal Paralysis (Ant. 

Cornual Myelitis) in the Adult. Lancet, November 14, 1885. 
Leclerc et Blanc. Paralysie spinale de l'adulte. Lyon m6d„ 1886, 52. 
Buss. Ein seltener Fall von atrophischer Spinallahmung (Poliom. ant. chron. 

adult.) mit Uebergang in acute Bulbarmyelitis. Berliner klin. Wochenschr., 

1887, No. 28. 



CHRONIC ANTERIOR POLIOMYELITIS. 



433 



Oppenheim. Ueber Poliomyelitis ant. chron. Deutsche Med.-Ztg., 1887, 95, 

p. 1087. 
Oppenheim. Arch. f. Psych, u. /Nervenkr., 1888, xix, 2, p. 381. 
Raymond. On Essential Myopathies. Gaz. des hop., 1888, 150. 
Hoffmann (Heidelberg). Ueber progressive neurasthenische Muskelatrophie. 

Arch. f. Psych, u. Nervenkr., 1889, xx, 3. 
Higier. Ueber primare und secundare Amyotrophien organischer und dy- 

namischer Natur. Deutsche med. Wochenschr., 1893, 37, 38. 



2S 



CHAPTER II. 

ATROPHIA MUSCULARIS PROGRESSIVA SPINALIS — PROGRESSIVE 
MUSCULAR ATROPHY. 

Progressive muscular atrophy was first described by Du- 
chenne and Aran in 1849 an d 1850, and was recognized by 
Cruveilhier in 1855 as a spinal affection. Thanks to the work 
of Lockhart Clarke, and especially that of Charcot, the occur- 
rence of a pathological process restricted to the gray substance 
of the spinal cord, which is accompanied by a muscular atrophy 
of typical distribution, is now established beyond the slightest 
doubt. 

Pathological Anatomy. — The process, which is usually most 
pronounced in the cervical cord, consists again of an atrophy 
and transformation of the gray anterior horns into a fine fibrous 
tissue containing spider cells. The large ganglionic cells are 
partly or wholly destroyed, or at any rate are diminished in 
number and perceptibly smaller. Here, too, the lesion extends 
to the anterior nerve roots and corresponding fibres of the 
motor nerves. On microscopical examination we find that the 
muscles supplied by them retain their transverse striation, but 
the fibres are decidedly diminished in size. Some fibres also 
show the so-called degenerative atrophy — that is, a fatty, wax- 
like degeneration, with increase of the interstitial connective 
tissue and multiplication of the muscle nuclei. Which of the 
described processes has to be regarded as the primary one, in 
other words, whether the disease actually does start in the 
gray matter of the cord, and not perhaps in the peripheral 
nerve endings ; whether both processes may occur at the same 
time, or whether they may succeed each other in the same 
individual, and at what age they occur, all these points have 
recently given rise to much controversy, as has also the ques- 
tion of the importance of hereditary influences. Those who 
wish to inform themselves more thoroughly on this subject 
are referred to the articles by Hoffmann (Deutsche Zeitschr. f. 
434 



PROGRESSIVE MUSCULAR ATROPHY. 



435 



Nervenheilk., 1893, iii, 6, p. 427), Striimpell (ibid., p. 471), Bern- 
hardt, Ueber die spinal-neurotische Form der Muskelatrophie 
(Virch. Arch., 1893, cxxiii, Heft 2), and others. 

Symptoms. — The onset of the disease is in many cases very 
characteristic. The patient begins to complain of weakness in 
the arms, sometimes more in the right than in the left, which 
soon interferes to some extent with his ordinary actions. Sen- 
sory changes and pains are absent — a point which is of vast 
diagnostic importance. Not many weeks after these symptoms 
have appeared the competent observer will notice a peculiar 
flatness, a sunken-in condition of the ball of the thumb, while 
at the same time the thumb is more than usually approximated 
to the second metacarpal bone (" ape hand," Fig. 138). The 
interosseal spaces on the back of the hand are sunken in and 





Fig. 138. Fig. 139. 

Figs. 138, 139.— Progressive Muscular Atrophy. (After Eichhorst.) Fig. 138, ape 
hand. Fig. 139, sunken-in interosseal spaces on the back of the hand. 

the terminal phalanges of the fingers are in incomplete exten- 
sion (Fig. 139). The hollow of the hand seems flattened (atro- 
phy of the lumbricales), and the atrophy of the muscles of the 
thenar and hypothenar becomes more and more apparent. As 
the function of the interossei becomes disturbed to a greater 
extent, the same claw-like position of the fingers develops 
which has been described on page 349 as occurring in affec- 
tions of the ulnar nerve (" claw hand," " main en griffe"). 

After this condition has thus for weeks or months under- 
gone no marked change, the disease begins to attack either the 
muscles of the forearm, or, passing over these, implicates the 



436 DISEASES OF SUBSTANCE OF SPINAL CORD. 

muscles of the shoulders and with special preference the del- 
toid. In the former case the extensors are attacked earlier and 
more seriously than the flexors. The muscles of the trunk and 
legs are either later or never affected, but if invasion of the 
diaphragm and other respiratory muscles occur this may prove 



Fig. 140.— Progressive Spinal Muscular Atrophy (personal observation). 

fatal, as may also an extension of the process from the cord to 
the medulla oblongata, in which case the symptoms of pro- 
gressive bulbar paralysis are superadded (page 154). If this 
does not take place and the respiratory muscles are spared, the 
disease may last for years and tens of years, and death is only 
caused by an intercurrent acute malady. 



PROGRESSIVE MUSCULAR ATROPHY. 



437 



Apart from the characteristic onset, the following signs 
help to make the diagnosis certain: (i) Fibrillary twitchings 
in the affected muscles, which can at times be produced by tap- 
ping the muscles, but which are often seen to appear of 'their 
own accord and continue without interruption. (2) The con- 




Fig. 141.— Progressive Spinal Muscular Atrophy .personal observation). 



dition of the electrical excitability, which depends directly on 
the number of muscle fibres left. If the greater number of 
the fibres are wasted, then the excitability for both currents is 
equally decreased. If all the fibres of a muscle have disap- 
peared and only fat and connective tissue remain, the excita- 
bility of the muscle is completely lost. It is only exceptionally 



438 DISEASES OF SUBSTANCE OF SPINAL CORD. 

that the excitability also undergoes qualitative changes and we 
find reaction of degeneration. (3) The loss of the tendon re- 
flexes, which is sufficiently explained by the disappearance of 
the ganglionic cells, a part of the reflex arc. It is only because 
the lower extremities are rarely affected that the patellar re- 
flexes are usually retained. (4) Sensibility remains everywhere 
and for all kinds of impressions intact (touch, pressure, pain, 
temperature). The coldness and blueness of the hands is to be 
attributed to the disuse of the muscles. True trophic disturb- 
ances of the skin, as well as bladder and rectal symptoms, are 
usually absent. 

Diagnosis. — Remembering, then, the different points just 
alluded to, the diagnosis should be easy, and it will not be 
difficult to avoid confounding the disease with myelitis, neu- 
ritis, or syringomyelia. The flaccid paralysis, the absence of 
all symptoms of motor irritation and sensory disturbances, is 
especially of moment in differentiating this disease from mye- 
litis. More particularly characteristic is the commencement, 
the onset of the disease in the small muscles of the hands. If 
this has been well pronounced, an error in diagnosis is unpar- 
donable. 

-^Etiology. — With reference to the aetiology a little more is 
known about this disease than about spinal infantile paralysis ; 
for certain cases at least it has been shown that overexertion 
of the muscles, as happens sometimes to those who work with 
the sewing machine, has a causative influence, or at any rate 
the disease has been preceded by some overexertion of the 
muscles, to which we are then justified in attributing an setio- 
logical importance. The conditions, however, under which 
paralysis and fatigue of the muscles lead to atrophy — why, for 
instance, the serratus magnus (Chvostek) is in some cases at 
first thus affected — we are wholly ignorant of, just as we do 
not know the conditions under which the genuine hypertrophy 
develops which we often find in the biceps of blacksmiths. 
Recently, again, attention has been called to the fact that the 
disease may be hereditary, by Bernhardt (Virchow's Arch., 
1889, 115, 2) and by Werdnig (Arch. f. Psych., xxii, 2). 

Little need be said about the therapeutics ; there is no 
effectual treatment, and all measures that have been tried 
have not been efficient in hindering the progress of the dis- 
ease. 



LESIONS OF WHITE MATTER OF SIINAL CORD. 439 



LITERATURE. 

Chvostek. Oesterr. Zeitschr. f. prakt. Heilk., 1871, xvii, 13-16. 

Lockhart, Clarke. Med.-Chir. Transact., 1873, lvi, p. 103. 

Charcot. Lectures on the Diseases of the Nervous System, delivered at the 
Salpetriere. Translated by George Sigerson. New Sydenham Society, 
London, 1877. 

Pierret et Troisier. Arch, de physiol., 1875, 2me ser., ii, 2. 

Bode. Casuist. Beitrage zur Aetiologie, Symptome und Diagnose der progress. 
Muskelatrophie. Inaug.-Dissert, Halle, 1881. 

Westphal. Charite-Annalen, 1886, xi, p. 357. (Progressive Muscular Atrophy, 
with Implication of the Facial Muscles.) 

Schultze, F. Ueber den mit Hypertrophic verbundenen progressiven Muskel- 
schwund und ahnliche Krankheitsformen. Wiesbaden, Bergmann, 1886. 

Landouzy et Dejerine. Nouvelles recherches cliniques et anatomo-pathologiques 
sur la myopathic atrophique progressive a propos de six observations nou- 
velles dont une avec autopsie. Revue de med., 1886, vi, 12, 977-1027. 

Charcot et Marie. Sur une forme particuliere d'atrophie musculaire progressive, 
etc. Ibid., 1886. vi, 2, p. 97. 

Ladame. Contribution a l'etude de la myopathic atrophique progressive. Ibid., 
1886, vi, 10, p. 817. 

Strumpell, A. Deutsches Arch. f. klin. Med., 1887, Bd. xlii, 1-3, p. 230. 

Bernhardt. Ueber einen Fall von (juveniler) progressiver Muskelatrophie mit 
Betheiligung der Gesichtsmusculatur. Berliner klin. Wochenschr., 1887, 
No. 41. 

Spillmann et Haushalter. Observation de myopathic progressive primitive a 
type facio-scapulo-humeral. Revue de m£d., 1888, vi. 

Raymond. Atrophies musculaires et maladies atrophiques. Paris, Doin, 1889. 

Sachs. The Peroneal Form or Leg-type of Progressive Muscular Atrophy. Brain, 
1890, xlviii, p. 447. 

Gombault. Sur l'etat des nerfs peripheriques dans un cas de myopathic pro- 
gressive. Arch, de med. experim., 1890, 1,4, 5. 

Bernhardt. Neuropathologische Beobachtungen. Zeitschr. f. klin. Med., xvii, 
Suppl.-Heft, 1889. 

Freyhan. Ibid., 4, 1891, xix. 

Bruns. Schmidt's Jahrbucher, 1894, ccxli, No. 3. 

II. LESIONS OF THE WHITE MATTER OF THE SPINAL CORD — 
" LEUCOMYELITIS." 

While, as we have said on page 424, the lesions affecting 
the gray matter (poliomyelitis) are almost entirely confined to 
one portion of it — namely, the anterior horns — we shall soon 
see that this is different with the lesions of the white matter, 
to which the general name leucomyelitis may be given (Xeu/co?, 
white). Here different parts can be attacked, either alone or 
in conjunction with others, and it is of great importance to 
differentiate between the clinical symptoms which occur in 



440 DISEASES OF SUBSTANCE OF SPINAL CORD. 

the diseases of the different columns or " systems " (Flechsig), 
hence called " system diseases." 

The affection is either a primary one, when it is often im- 
possible to ascertain any ^etiological factor, or it occurs sec- 
ondarily and as a consequence of certain affections of the 
brain and the spinal cord itself, such as traumatic inflamma- 
tions and compression. We shall consider both separately. 

A. Primary Lesions of the White Columns. 

Regarded from an anatomical standpoint, the primary tract- 
degenerations of the white substance consist in a destruction of 
the nerve fibres and a simultaneous increase of the neuroglia. 
The medullary sheaths are the first to disappear ; the axis cylin- 
ders, which are more resistant, do not degenerate till later. 
Compound granular corpuscles, which remove the detritus 
from the diseased regions (Ziegler), accumulate in the lymph 
sheaths of the vessels. The increasing neuroglia crowds in 
and displaces the empty nerve tubes, a process which, in con- 
junction with the thickening of the walls of the vessels, which 
develops at the same time, is described under the name of scle- 
rosis, or gray degeneration. 

An affection confined to one nerve tract or system has up 
to this time only been observed in the antero-lateral but not in 
the posterior columns. In the former, the anatomical arrange- 
ment of which has been described above, we meet especially 
frequently with sclerosis of the so-called crossed pyramidal 
tracts, but the lesion does not necessarily extend over the 
whole length of the tract, but may be only partial (Westphal). 
Most of the cases which have come under observation were, 
however, not pure instances, but presented other anatomical 
changes as well, and there is only one case reported, by 
Dreschfeld, in 1881, which, viewed from an anatomical stand- 
point, can pass for a pure lateral sclerosis. 

The primary sclerosis of the lateral columns — spastic spinal 
paralysis, tabes dorsale spasmodique — was first described by Erb 
and Charcot in 1875, and characterized by them as a motor 
paralysis with remarkable increase in the tendon reflexes; and, 
indeed, if we examine such patients, all we find is that they 
have lost to a greater or lesser extent the use of their legs ; 
they are unable to walk, the feet are glued, as it were, to the 
floor, and the patient can only shuffle along, the inner margin 
of the foot never leaving the ground. At the same time the 



PRIMARY LESIONS OF THE WHITE COLUMNS. 



441 



muscles feel firm and hard, the legs are in extension, and any 
attempt at flexion is difficult. If such a patient is made to sit 
on the edge of a table the legs do not hang down flaccidly, as 
might be expected, but are thrown into a state of tetanic 
tremor, produced by contractions of the quadriceps extensor. 
There is an enormous exaggeration of the patellar reflexes, and 
the ankle clonus is obtained without the slightest difficulty. 
With the exception of the inability to walk, the patient has no 
subjective complaints ; neither sensation nor the functions of 
the bladder, rectum, or the sexual apparatus show any abnor- 
mity. An implication of but one of these would at once 
exclude the diagnosis of lateral sclerosis, as would also (and 
this should be especially remembered) the existence of any 
muscular atrophy. The very characteristic spastic or spastic- 
paretic walk of the patient, the traces which his feet leave on 
a gravel path, for instance, and which can be followed up as 
distinct continuous streaks, the shuffling noise which accom- 
panies every step when he attempts to walk about the room, 
these are of great diagnostic value ; the examination of the 
soles of the patient's shoes, which appear thinner and more 
worn down on the inner side, will be of interest and value. 

The disease may be congenital (Lorenz, Bernhardt, cf. lit.), 
and may occur in more than one member of the same family, 
as we have stated above (page 274), but it often begins later in 
youth or in middle life, attacking first the one then the other 
leg, without, as a rule, extending to the arms or trunk, yet the 
upper extremities are said to be occasionally affected (Strum- 
pell). The disease may last years or tens of years without pre- 
senting any decided change for the worse. Death is brought 
about by intercurrent diseases. It is not known whether, as in 
progressive muscular atrophy, overexertion is of astiological 
importance ; instances, however, in which acrobats (Donkin) 
and hod-carriers (Munter) were attacked seem to suggest 
this. Morgan pointed out that exposure to cold, such as long 
standing in water, may be the immediate cause of the disease 
(Morton, Lancet, January 19, 1881). 

The form of spastic paralysis, analogous to a tabes devel- 
oping on a syphilitic basis, which has been regarded by Charcot 
as a transverse syphilitic myelitis, and which has been studied 
carefully first by Erb, later by Muchin, P. Marie, and Kowa- 
lewsky (Neurol. Centralbl., 1893, 12), must be regarded as a dis- 
tinct disease. It occurs much less frequently than tabes, and 



442 DISEASES OF SUBSTANCE OF SPINAL CORD. 

differs from the spinal paralysis just described, inasmuch as here 
we find sensory and trophic changes as well as eye-muscle pal- 
sies. The differential diagnosis may, however, be impossible. 

Much more frequent than a lesion confined to the crossed 
pyramidal tracts is one which implicates not only these, but 
with them the posterior columns and the direct cerebellar 
tracts, in which, although not always, Clarke's columns take 
part. The anatomical character of this "combined system 
disease " which results from these lesions has been repeatedly 
described (Westphal, Gowers, Strumpell). The symptoms 
vary according to the distribution of the lesion ; thus, if the 
disease of the lateral columns extends low down, while the 
posterior columns in the dorsal and lumbar region present no 
changes, rigidity of the muscles and increase of the reflexes 
will be found. If, on the other hand, the disease in the poste- 
rior columns extends farther downward, these symptoms will 
be absent, the lesion in the lateral being neutralized, as it were, 
by that in the posterior columns (Westphal). 

Not rarely the affection seems to depend on faulty develop- 
ment, a condition which we may meet with in more than one 
member of the same family, and which may be hereditary. In 
these cases the disease appears in early childhood, and, as we 
said, sometimes in several children of the same family. It has 
been called, after the author who first described it, Friedreich's 
" hereditary ataxia." Senator (cf. lit.) has called attention to 
the possibility of a congenital atrophy of the cerebellum, the 
medulla oblongata, and the spinal cord. The motor disturb- 
ances in the children begin in the feet, the walk becomes awk- 
ward, they stumble, and in passing over small obstacles have 
to look at their feet to keep from falling, etc. (Fig. 142). The 
patellar reflexes disappear ; the arms are not affected until 
later, and, indeed, they are by no means always implicated. 
The second motor disturbance establishes itself in the muscles 
of the tongue and the lips which are necessary for speaking, 
and this produces a very characteristic defect of speech of mo- 
tor origin. Finally the muscles of the eyes become implicated, 
and there results a distinct nystagmus. The combination of 
these three symptoms is pathognomonic for this rare disease. 
It has no connection with tabes and sensory changes, and blad- 
der symptoms, manifestations which are probably never want- 
ing in cases of tabes, are never met with in the disease under 



FRIEDREICH'S DISEASE. 



443 



consideration. Nor can it be mistaken for multiple sclerosis, 
as vertigo and " scanning speech " are never associated with 
it. The course is tedious, the prognosis always unfavorable, 
the muscles undergo atrophy in consequence of inactivity, and 
contractures occur in the joints. 

Similar symptoms are observed in adults in cases of com- 
bined lateral and posterior sclerosis ; yet there are certain 
peculiarities to which Gowers especially has drawn attention. 




Fig. 142. — Friedreich's Disease. The patient is held under the arms. 
(After Chauffard.) (Semaine med., 18^3, No. 32.) 

The disease was named by him " ataxic paraplegia." The 
lower extremities are ataxic and paretic, which gives rise to an 
uncertain, swaying walk ; but this is associated with parassthe- 
sias, weakness of the sphincters, and decrease of the sexual 
power. The patellar reflexes are at first increased, and only 
at times become lost later in the disease. Such an increase is 
never known in hereditary ataxia. During the period of in- 
crease, rigidity of the muscles, spasm, and ankle clonus are 
present. It is evident that cases of this kind may be mistaken 



444 DISEASES OF SUBSTANCE OF SPINAL CORD. 

for tabes, especially if the patellar reflexes are absent, as excep- 
tionally occurs. Then the history may be of use to us, as 
syphilis seems to possess no ^etiological importance whatever 
in the combined sclerosis, while exposure to cold and over- 
exertion seem to be of considerable moment. 

LITERATURE. 
Spastic Spinal Paralysis. 

Leyden. Arch. f. Psych, u. Nervenkrankh., 1878, viii, 2, p. 761. (Experi- 
mentally Produced Spinal Sclerosis.) 

Mdbius. Zur spastischen Spinalparalyse. Schmidt's Jahrb., 1880, Bd. clxxxviii, 
p. 129. (Many references.) 

Donkin. Brit. Med. Journ., December 9, 1882. (Spastic Paraplegia in an 
Acrobat.) 

Westphal. Ueber einen Fall von sog. spastischer Spinalparalyse mit anat 
Befunde. Arch. f. Psych, u. Nervenkrankh., 1884, xv, 1, 224. 

Pitres. Un cas de paralysie generate spinale anterieure subaigue suivi d'autop 
sie. Progr. med., 1888, 35. 

Knuth. Ueber spastische Spinalparalyse mit Dementia paralytica. Kiel, 1888 

Richardson. Case of Infaniile Spastic Paralysis. Lancet, November, 1888 
ii, 19. 

Bloch. Beitrage zur Pathologie der spastischen Spinal paralyse. Inaug.-Dissert. 
Berlin, 1890. 

Feer. Ueber die angeborene spastische Gliederstarre, Mittheil. aus dem Kin- 
derspital zu Basel. Jahrb. f. Kinderheilk., 1891, pp. 216-290. 

Lorenz. Ueber angeborene spastische Paralyse. Deutsche Med.-Ztg., 1891,90. 

Williamson. The Changes in the Spinal Cord in a Case of Syphilitic Paraplegia ; 
Sclerosis of the Lateral Pyramidal Tracts, and Goll's Columns with Pe- 
ripheral Sclerosis. Medical Chronicle, Manchester, 1891, xiv, pp. 269-276. 

Erb. Ueber syphilitische Spinalparalyse. Neurol. Centralbl., 1892, 6. 

Friedreich 's Disease. 

Brousse. De l'ataxie hereditaire. Paris, 1882. (Maladie de Friedreich.) 

Rutimeyer. Virchow's Arch., 1883, Bd. xci, 2. 

Erlenmeyer. Centralbl. f. Nervenheilk., 1883, vi, 17. 

Walle. Schweizer Correspondenzbl., 1884, xiv, 2. 

Musso. Riv. clin., 1884, xxiii, 10. 

Longuet. L'Union, 1884, 72. 

Schulze, F. Arch. f. Psych, u. Nervenkrankh., 1884, xv, 1, p. 262. 

Seguin. New York Med. Record, 1885, xxvii, 29. 

Sinkler. Journ. of Nerv. and Ment. Diseases, 1885, xii, 3. 

Ormerod. Med.-Chir. Transact., 1885, lxviii, p. 147. 

Judson, S. Bury. Brain, July, 1886, ix. 

Stintzing. Munch, med. Wochenschr., 1887, Bd. xxxiv, 21. 

Charcot. Progr. med., 1887, 23. 

Rutimeyer. Ueber hereditare Ataxic Virchow's Arch., 1887, no, 2. 

Ormerod. Brain, 1888, xxxix and xl. 

Gilles de la Tourette. Nouv. iconograph. de la Salpetr., 1888, 3. 



SECONDARY LESIONS OF THE WHITE COLUMNS. 



445 



Ladame. La maladie de Friedreich. Geneve, Schuchardt, 1889. 

Dejerine et Letulle. La maladie de Friedreich. Med. Moderne, 1890, i, 17, p. 

33i. 

Menzel. Arch. f. Psych, u. Nervenkrankh., 1890, p. 160. 

Blocq et Marinesco. Arch, de Neurol., 1890, xix, No. 57. 

Mott. Complete Sclerosis of Goll's Columns and Chronic Spinal Leptomen- 
ingitis, with Degenerative Changes in the Fibres of the Anter. and Post. 
Roots. Amer. Journ. Med. Sciences, ci, January, 1891. 

Geigel, R. Ueber hereditare Ataxic Sitzungsbericht der Wiirzburger phys.- 
med. Gesellschaft, 1892. 

Senator. Berliner klin. Wochenschr., 1893, 21. 

Combined System Diseases. 

Kahler und Pick. Arch. f. Psych, u. Nervenkrankh., 1877, viii, p. 251. 

Prevost. Arch, de physiol., 1877, 2me ser., iv, 3, 4, 5. (Combined Sclerosis of 
the Posterior and Lateral Tracts.) 

Striimpell. Arch. f. Psych, u. Nervenkrankh., 1880, xi, 1. 

Edes. The Somewhat Frequent Occurrence of Degeneration of the Postero- 
lateral Columns of the Spinal Cord in so-called Spinal Concussion. Boston 
Med. and Surg. Journ., September 21, 1882. 

Grasset. Du tabes combine (ataxo-spasmodique) ou sclerose postero-laterale de 
la moelle. Arch. de. Neurol., 1886, xi, xii. 

Gowers. Ataxic Paraplegia. Lancet, 1886, ii, 1, 2, 

Babinski et Charrin. Sclerose medullaire systematique combinee. Revue de 
med., 1886, iii, 11, p. 962. 

Striimpell. Ueber eine bestimmte Form der primaren combinirten Systemer- 
krankung des Riickenmarks. Arch. f. Psych., etc., 1886, xvii, 1. 

Erlicki et Rybalkin. Zur Frage liber die combin. Systemerkrankungen des 
Riickenmarks. Ibid., 1886, xvii, 3. 

Dana. Progressive Spastic Ataxia (Combined Fascicular Sclerosis) and the 
Combined Sclerosis of the Spinal Cord. The Med. Record, July 2, 1887. 

Adamkievvicz. Wiener med. Wochenschr., 1888, 17. 

Kiewlicz. Arch. f. Psych, u. Nervenkrankh., 1889, xx, 1. (Myelitis Trans- 
versa, Syringomyelia, Multiple Sclerosis, and Secondary Degenerations.) 

Francotte. Etude sur l'anatomie pathologique de la moelle epiniere. Arch, de 
Neurol., 1890, xix, 57. 

B. Secondary Lesions of the White Columns. 

Lesions of the motor centres of the brain cortex, or lesions 
of the path between these centres and the motor ganglia of the 
spinal cord, the so-called cortico-muscular tract or pyramidal 
tract, give rise to a descending degeneration of the motor fibres 
on the same side as the brain lesion. This secondary degen- 
eration is in the cord continued in the crossed pyramidal tract 
of the opposite side, while the direct pyramidal tract presents 
only traces of it. About the causes of this sclerosis we possess 
just as little definite knowledge as about the clinical symptoms 



446 



DISEASES OF SUBSTANCE OF SPINAL CORD. 



by which it manifests itself. The former is sought in the cut- 
ting off of the parts from their trophic centres, and with regard 
to the latter it is generally supposed that 
the gradual developing rigidity of the mus- 
cles, the increase of the reflexes, and the 
later contractures depend on this degen- 
eration. The cases, however, in which at 
the autopsy an exten- 
sive degeneration was 
found, while during 
life not a trace of such 
symptoms was pres- 
ent, do not speak much 
in favor of this view. 

Lesions of the whole 
transverse section of 
the cord also produce 
secondary degenera- 
tion, which, however, 
extends not only down- 
ward (in the pyrami- 
dal tracts), but also up- 
ward— (i) in the inner 
segment of the poste- 
rior columns (Goll, cf. 
Fig. 143), and (2) in the 
direct cerebellar tracts 
(Flechsig), which are 
in connection with 
Clarke's columns (cf. 

Fig. 144). While this ascending degener 
ation is physiologically extremely interest- 
ing, as it indicates that the trophic centres of these two tracts 
must be situated more peripherally (as, for instance, in Clarke's 
columns), we are not as yet able to attribute any clinical im- 
portance to it. 





Fig. 143.— Ascending and 
Descending Degener- 
ation in the Spinal 
Cord. A, primary area 
of degeneration (lesion). 
B, degeneration of Goll's 
columns (ascending). C, 
degeneration of the 
crossed pyramidal tract 
(descending). (After 

Gowers.) 



Fig. 144.— Secondary As- 
cending and Descend- 
ing Degeneration in 
a Transverse Affec- 
tion of the Upper 
Dorsal Cord. The as- 
cending degeneration oc- 
curring in Goll's columns 
and the direct cerebellar 
tracts, the descending de- 
generation in the crossed 
pyramidal tracts. (After 
Strumpell.) 



III. LESIONS OF THE GRAY AND WHITE MATTER OF THE 
SPINAL CORD. 

Charcot and Joffroy were the first to show that the large 
ganglionic cells of the gray anterior horns and the pyramidal 
tracts can be affected simultaneously by a disease which pro- 



AMYOTROPHIC LATERAL SCLEROSIS. 



447 



duces characteristic clinical symptoms, but it was not until 
Flechsig announced his discovery of the system of conducting- 
fibres that these clinical observations became fully understood. 
Now we know that the disease which the French authors, fol- 
lowing Charcot, have termed sclerose laterale-amyotropJiiqiie — 
amyotrophic (more properly myo-atrophic) lateral sclerosis — 
consists of a lesion of the cortico-muscular tract, which begins 
as a degenerative atrophy in the lumbar cord, and which, as 
Charcot and Marie, and more recently Rott and Mouratoff 
(Moscow, 1890), have pointed out, can be traced as far as the 
motor nerve cells of the central convolutions. Attention has 
already been called to the fact that, just as the nerve cells 
of the anterior horns, in the same way the motor nuclei of the 
medulla oblongata may be implicated, and thus the clinical 
picture of progressive bulbar paralysis develop. The two dis- 
eases are therefore analogous, and akin to them is a third — 
namely, the progressive spinal muscular atrophy — in which 
affection also the large nerve cells are diseased, as we have 
already pointed out above. From the nerve cells the atrophy 
spreads toward the periphery to the anterior nerve roots and 
the muscles supplied by them. 

That the clinical manifestations are strictly motor and 
trophic, and that no sensory changes can occur, we can well 
understand from the anatomical distribution of the lesion. 
The patients at first complain of weakness in the arms and the 
hands, which soon interferes with their occupation. This loss 
of strength increases fairly rapidly, and the atrophy in the 
muscles of the hand — the thenar, the antithenar, and interossei 
— becomes more and more apparent. 

The muscles of the arms also waste, more especially those 
of the extensor side, and the former roundness of the shoulder 
is soon lost owing to the atrophy of the deltoid. The triceps 
and other muscles also then take part in the lesion, and the 
helplessness of the patient, who has but little use of his up- 
per extremities, rapidly increases. At the same time the ten- 
don reflexes are increased, and tapping of the bones of the 
forearm elicits lively contractions of the muscles (" periosteal 
reflex "). 

That the so-called "jaw-jerk," which has been described by 
De Wattewille, is characteristic of the disease I am very much 
inclined to doubt, since in a number of perfectly healthy per- 
sons I found it in some present, in some absent. It certainly 



448 DISEASES OF SUBSTANCE OF SPINAL CORD. 

does not possess any diagnostic value. This jerk may be pro- 
duced by pressing down the lower jaw by means of a broad 
paper-cutter and tapping the latter with a percussion hammer 
near the teeth. The lower jaw will then respond with a con- 
traction of the muscles of mastication. 

In a relatively short time the paralysis of the upper ex- 
tremities becomes so complete that not even the slightest motion 
is possible, and gradually contractures develop (by preference 
in the wrist and elbow joint). In the lower extremities the 
same changes may be noted, but they make their appearance 
later and do not reach such a high degree. Here, too, we 
have first weakness, difficulty in walking, and general awkward- 
ness in making movements, then rigidity and stiffness of the 
muscles, enormously increased patellar reflexes and ankle clo- 
nus, later on total immobility and contractures in hip, knee, 
and ankle joints. 

A case in one of my wards, a woman thirty-four years of 
age, has been for two years without power of motion, and is so 
entirely deprived of the use of her four extremities that with- 
out assistance she is unable to make even the slightest motion 
with either fingers, hands, arms, toes, feet, or legs. The dis- 
ease goes on to invade the motor nuclei of the medulla oblon- 
gata, and hence is produced difficulty in swallowing, which 
ultimately amounts to a total inability to get food down, and 
the patient dies of starvation. At other times a disturbance of 
the respiratory apparatus may bring about a fatal issue. It is 
exceptional that the whole course of the disease comprises a 
period of more than two or three years. The diagnosis is not 
always easy, though it is not difficult to differentiate the disease 
from progressive muscular atrophy if its duration and the con- 
dition of the reflexes are borne in mind. But it is not always 
possible to decide between this and hysterical conditions — for ex- 
ample, the hysterical amyotrophia — as Charcot showed shortly 
before his death (Arch, de Neurologie, 1893, xxv, 74). Of the 
cause of the disease, as well as of effectual means wherewith to 
combat it, we are equally ignorant. 

LITERATURE. 

Charcot et Marie. Arch, de Neurol., 1885, x, 28, 29. 
Kojewnikoff. Centralbl. f. Nervenheilk., 1885, viii, 16. 
De Wattewille. Neurol. Centralbl., 1886, v, 3. (Jaw-jerk.) 
Rybalkin. Centralbl. f. Nervenheilk., 1886, ix, 8. (Jaw-jerk.) 



TRANSVERSE MYELITIS. 



449 



Zacher. Neurol. Centralbl, 1886, v, 23. (Amyotrophic Lateral Sclerosis Com- 
plicated by Dementia Paralytica.) 

Marie. Observations de sclerose laterale, amyotrophique, etc. Arch, de Neurol., 
1887, xiii, p. 387. 

Musso. Rivista clinica, June, 1887. 

Lennmalm. Upsala lakareforen. Forh., 1887, xxii, 7. 

Florand. Contribution a l'£tude de la sclerose laterale amyotrophique. These 
de Paris, 1887. (" Maladie de Charcot") 

Rovighi e Levi. Contributione alio studio della Sclerosi laterale amiotrofica. 
Reggio Emilia, 1888. 

Risse. Deutsches Arch. f. klin. Med., 1889, xliv, 5, 6, p. 523. 

Joffroy et Achard. Note sur un cas de sclerose laterale amyotrophique. Arch. 
de Med. experim. et d'Anat. path., 1890, pp. 434-446. 

While the diseases of the cord which we have studied so 
far were confined to certain s} r stems of fibres — in other words, 
were " system diseases " — the affection now to be considered 
does not present this peculiarity, but the process which affects 
the gray as well as the white matter is more or less widely ex- 
tended over the cross-section of the cord, forming- a small 
number of large or numerous small foci. In other words, the 
disease is what we call " asystemic " or diffuse. It is an inflam- 
mation of the cord, which according to its course is called an 
acute or chronic myelitis, and to which the name transverse 
myelitis has also been given. 

Pathological Anatomy. — Anatomical changes may in such 
cases be scarcely demonstrable even though the severest para- 
lytic symptoms may have existed during life. This is more 
especially true in cases of spinal paralysis due to pressure, 
occurring in consequence of disease of the vertebrae. Here 
we must assume that even moderate pressure is capable of 
bringing about a break in conduction without any destruction 
of nerve elements. Usually in cases where changes can be 
demonstrated we find a diminution in the size and an atrophy 
of the nerve fibres. The axis cylinders may appear swollen 
and may have lost their myeline sheath. The nerve cells, 
which are not affected until later, become shrunken and lose 
their processes. According to Friedmann, the degeneration be- 
gins in a circumscribed portion of the cell, secondarily the 
nucleus and the processes degenerate, and finally the whole 
cell shrinks or disintegrates (Neurol. Centralbl., 1891, 7; cf. 
also Fiirstner and Knoblauch, Arch. f. Psych., 1891, xxiii, 1). 
While thus the nerve tissue undergoes disintegration the sup- 
porting tissue increases, the meshes of the neuroglia become 
29 



45<D DISEASES OF SUBSTANCE OF SPINAL CORD. 

broader, and in it are seen the cells of the supporting tissue 
first described by Deiters, which, owing to their numerous pro- 
cesses, have also been called spider cells. In the meshes of the 
neuroglia reticulum compound granular corpuscles are found 
which have taken up the fat and disintegrated nerve substance. 
These are leucocytes, and in turn undergo, sooner or later, de- 
struction. The vessels are dilated and changes are seen in 
their walls, consisting of thickening or hyaline degeneration. 
In cases where this process has run its course in a compara- 
tively short time the cord is found at the autopsy to be soft 
and of a grayish-red color, whereas if the process has been slow 
the cord appears, in consequence of the increase of the support- 
ing tissue, hardened, or, as we say, " sclerosed." 

Macroscopically, little is to be seen. At the most some 
portions may, when the cord is put into Miiller's fluid for the 
purpose of hardening it, look light yellow, while others are 
dark green. The former are the diseased parts, which can not 
become stained because the myeline sheaths, which are turned 
green by chromium, are absent. With this exception all in- 
formation about the pathological changes must be derived from 
the microscopical examination of fresh as well as of hardened 
sections. 

According to the location of the process we distinguish a 
dorsal myelitis, the most common; a lumbar myelitis, the 
rarest; and a cervical myelitis, a relatively frequent form. In 
the first and second the upper extremities are entirely intact, 
while they are implicated if the process is situated in the cer- 
vical cord. 

Symptoms. — It is very natural that the clinical manifesta- 
tions of myelitis should, on the whole, very much resemble 
those which we have learned to recognize in the "system-dis- 
eases," and, as a matter of fact, almost all that will be described 
has already been said. Here, as there, we have to do with mo- 
tor, sensory, and trophic disturbances, with changes in the 
reflexes and symptoms referable to the bladder and rectum. 
The motor disturbances may consist of symptoms of paralysis 
and irritation. The former are usually the more prominent of 
the two, and weakness of the legs, which sooner or later 
amounts to complete palsy, is one of the chief symptoms of a 
myelitis. As a rule, both legs are about equally affected — 
paraplegia ; sometimes one retains its strength longer than the 
other, according to the extent to which the pyramidal tracts 






TRANSVERSE MYELITIS. 



451 



are diseased. If not the legs but the arms are paralyzed, the 
lesion is situated in the cervical cord. The symptoms of irri- 
tation consist of twitchings, which occur sometimes spontane- 
ously, sometimes as the result of slight stimulation of the skin. 
In many instances the removal of the bedclothes and the 
change of temperature resulting therefrom are sufficient to 
cause quite protracted clonic spasms of one or both legs. This 
and similar phenomena seem to be of reflex origin. 

The sensory changes are less regularly met with and are of 
less importance than the motor disturbances. There are in- 
deed cases where they are almost entirely absent, or where they 
at least do not annoy the patient or do not become marked 
until relatively late in the course of the disease. They consist 
mostly of paresthesias, numbness, formication, also of decrease 
in sensibility, which may amount to a complete anaesthesia, 
varying in extent and situation. Actual pains, which are suffi- 
cient from their duration and intensity to cause much suffering 
to the patient, and which are so commonly seen, as we shall 
learn, in tabes, belong in this disease to the exceptions. In 
fact, we may say that they are usually absent, or, at any rate, 
not at all severe. If we are able to detect sensory changes on 
the trunk itself, the level up to which these extend gives us 
valuable indications as to the seat of the myelitis. If it is in 
the lumbar cord, sensibility is intact above the navel ; if in 
the lower dorsal, above the middle of the sternum. Sensory 
changes in the neck and upper extremities indicate the seat to 
be in the cervical cord. The more prominent the sensory dis- 
turbances and the pains, the greater is the extent to which the 
gray matter of the posterior horns and the posterior columns 
participates in the inflammation or degeneration. 

Trophic disturbances appear when the trophic centres — 
that is, the ganglia of the anterior gray horns — are diseased. 
Thus, if we are able to demonstrate atrophy, with reaction of 
degeneration in the legs, this denotes a lesion of the gray an- 
terior horns in the lumbar cord, while the same condition in 
the arms indicates a disease of the anterior horns in the cervi- 
cal cord. The electrical examination should never be omitted 
in such cases, because it may happen that the legs present a 
certain degree of atrophy without the presence of any reaction 
of degeneration. This atrophy is, then, purely the result of 
disuse — the atrophy of inactivity. Other trophic disturbances 
or vaso-motor changes in the skin are not the rule. Herpes 



452 DISEASES OF SUBSTANCE OF SPINAL CORD. 

and urticarial eruptions, slight oedema and changes in the sweat 
secretion occur, but possess neither diagnostic nor prognostic 
value. 

One symptom remains still to be mentioned, because it is 
rarely wanting, but rather plays an important role in myelitis, 
and causes endless annoyance and discomfort to the patient — 
namely, the bed-sores which occur in the sacral region, and 
become the more extensive the less the care exercised in the 
nursing and for the cleanliness of the patient. This is one 
of the most important trophic disturbances, and one which, 
even with the most careful attention, can not in all cases be 
avoided. 

The condition of the skin as w T ell as the tendon reflexes 
depends (i) on the state of the reflex arc in the spinal cord, (2) 
on the state of the fibres coming from the brain, which have 
probably an inhibitory function. If the reflex arc is normal, 
but the conduction of the inhibitory fibres interrupted, then 
the corresponding reflex is increased, while if the reflex arc is 
diseased the reflex is lost, no matter whether the conduction 
of the inhibitory impulses be intact or not. This holds for the 
skin as well as tendon reflexes. Therefore in cases of lumbar 
myelitis not only the skin but also the tendon reflexes are 
diminished or lost in the lower extremities. Those concerned 
are the patellar reflex, the reflex arc of which corresponds to 
the cord between the second and fourth lumbar nerves ; the 
tendo-Achillis reflex, the arc of which corresponds to the first 
sacral nerve ; the cremasteric and abdominal reflexes which 
have their arc at the level of exit of the first lumbar and a por- 
tion of the cord between the fourth and seventh dorsal nerves 
respectively. On the other hand, in a dorsal or cervical mye- 
litis a marked increase of the tendon and skin reflexes of the 
lower extremities takes place, because the (supposed) inhibi- 
tory influences are cut off. 

A symptom which, perhaps, causes the patient himself more 
annoyance than any other is the disturbance in the functions of 
the bladder, which in a myelitis is hardly ever totally absent. 
At first there is some difficulty in micturition, which may end 
in complete retention, so that the patient can not void his 
urine, but requires to be catheterized. In the later stages of 
the disease, however, the urine is passed involuntarily, there 
being either a constant dribbling (incontinentia urinse) or from 
time to time an involuntary evacuation of the bladder. In 



TRA NS VER SE M YE LITIS. 



453 



either case the patients can not dispense with a portable urinal. 
Occasionally there is a painful burning sensation when the urine 
is passed (ischuria) so that the patient dreads every evacuation 
of the bladder. As might be expected, cystitis frequently 
develops in these cases, partly owing to the length of time that 
the urine remains in the bladder, partly owing to the frequent 
use of the catheter. The rectal symptoms consist either of a 
most obstinate constipation, or, if the sphincter ani becomes 
paralyzed, of incontinence of faeces (incontinentia alvi), which 
aggravates to a very serious extent any bed-sore that may be 
present. For the localization of the myelitic process neither 
bladder nor rectal symptoms can be used. They are always 
present at whatever level the lesion may be. 

Etiology. — Of the aetiology of myelitis little is known. It 
seems justifiable, however, to divide the causes into those 
which act chemically and those which act mechanically, the 
former being either of an infectious or of a toxic nature. That 
infectious diseases may produce myelitis is shown by the fact 
that it occurs occasionally after diphtheria and gonorrhoea 
(Leyden, cf. lit.), more frequently after small-pox, and also dur- 
ing the course of syphilis, and that the influence of poisons may 
at least favor the development of myelitis has been upheld 
since the action of arsenic, of mercury, and of lead, and the 
symptomatology of the resulting intoxications have been more 
accurately studied. Leyden has recently published studies 
upon the relation between grave anaemias and some forms of 
chronic myelitis ; Eisenlohr, upon the connection of primary 
atrophy of the mucous membrane of the stomach and intestines 
and myelitis. 

Among the mechanical causes the most important is pres- 
sure, which can be exerted upon the cord by structures sur- 
rounding it, as happens, for instance, in spinal meningitis and 
meningeal tumors. Of greater importance in this connection 
is the chronic caries of the vertebrae (malum Pottii), spondyl- 
arthrocace, the tubercular spondylitis, and carcinoma of the 
vertebrae (cf. Figs. 145 and 146), in which either the dislocated 
(diseased) vertebrae themselves or the caseous and inflamma- 
tory products which are found between the dura and the bone 
may exert a compressing influence. That there are still other 
causes which may give rise to myelitis we do not deny ; we 
would only mention bodily fatigue and exposure to cold, but 
these are infinitely rarer. On the other hand, there exists not 



454 



DISEASES OF SUBSTANCE OF SPINAL CORD. 



the smallest ground for the assertion that sexual excesses ever 
produce it. 

Course. — The course in general is the following : After the 
patient has for weeks and months managed with difficulty to 
get around, his legs becoming weaker and weaker, he has to 
take to bed or to the rolling chair, where he spends one, two, 
even four years, harassed by various afflictions, among which 
the bladder symptoms and the motor disturbances are espe- 
cially prominent. Recovery, if it occurs at all, is only very 
exceptional, and the prognosis must therefore always be very 




Fig. 145. Fig. 146. 

Complete Interruption of Conduction of the Spinal Cord during Life. Fig. 
145, anterior, Fig. 146, posterior aspect of the spinal cord. The dura mater is divided 
and folded back. Circular compression and narrowing of the spinal cord at K in conse- 
quence of carcinoma of the vertebrae in a woman thirty-four years of age. Natural size. 
The drawing is made from a fresh preparation. (After Eichhorst.) 

unfavorable. Death occurs in consequence of bed-sores, which 
are seldom absent, or is at least precipitated by them. Some 
patients die from intercurrent diseases, others from the cystitis. 
The course of the so-called pressure myelitis and its resulting 
pressure paralysis, the symptoms of which have before been 
alluded to on page 424, is so far characteristic that we can 
here distinguish a prodromal stage, a stage of irritation, and a 
stage of paralysis. The prominent features of the first are 
rigidity of the vertebral column, dull, vague pains in the back, 
and the first signs of a commencing deformity. In the second 
stage we have severe neuralgic pains, hyperaesthesias, parak- 
inesias, and girdle sensations. In the third, finally, paralytic 
symptoms, increased reflexes, vaso-motor and trophic disturb- 
ances (herpetic eruptions, muscular atrophies, bed-sores, etc.). 



TRANSVERSE MYELITIS. 455 

This distinction, however, is only possible in isolated cases. 
Bladder and rectal symptoms are absent in no case of pressure 
paralysis. 

In our prognosis we must not leave out of consideration 
the possibility that the inflammatory new formations in the 
vertebrae may disappear, and thus, the cause which produced 
the break in the conduction ceasing to act, it may be possible 
for the spinal cord to recover completely all its normal func- 
tions, provided, of course, that none of the nerve elements have 
been destroyed. 

Treatment. — The treatment of any case of myelitis necessi- 
tates much patience on the part of the sufferer, because weeks 
and months may pass before any sign of improvement can be 
perceived, and much circumspection on the part of the phy- 
sician, because we are never able to say beforehand how cer- 
tain measures are going to be borne by the patient, and because 
what often helps one is harmful to another ; hence one must 
proceed carefully and systematically, and as it is likely that 
the course of the disease is going to extend over years, one 
should always have something new and as yet untried in re- 
serve. If the diagnosis has once been made with certainty, it 
is our duty to inform the patient in a delicate way of the true 
state of affairs, and how seriously his capacity for following his 
occupation will be interfered with ; further, to see that he is 
properly fed on a nourishing diet, and obtain for him as far as 
possible mental and bodily rest. It is a gross error to recom- 
mend such patients, who are easily fatigued and who on the 
slightest provocation are attacked by all sorts of pains, to take 
as much exercise as possible, or even to prescribe gymnastics 
for them. 

The electrical treatment is indicated and ought to be begun 
early. The constant current should be applied near the seat 
of the lesion (the anode being placed on the tender parts of the 
spinal column if there be such), the faradic to the peripheral 
parts, especially the lower legs. Definite rules can not be laid 
down. It is best to seek information from a reliable text-book, 
and to try which mode of treatment is best borne by the pa- 
tient and by which most is accomplished. Tepid baths — 84 
to 88° Fahr. — three or four times a week for from fifteen to 
thirty minutes, best taken in the forenoon, usually have a favor- 
able influence, and are, if not of lasting benefit to the patient, 
frequently productive of at least a transient feeling of comfort. 



456 DISEASES OF SUBSTANCE OF SPINAL CORD. 

The addition of rock-salt, sea-salt, or lye (one or two quarts) 
should only be ordered if the patient himself seems to lay much 
stress on it, as we can not expect any especial effect from 
them. Neither should we raise our expectations too high 
when we recommend warm brine baths containing carbonic- 
acid gas, or non-medicated warm baths, or mud baths and the 
like. Of course every patient, rich or poor, expects us to send 
him in summer to the springs, but he will gradually find out 
that the success attained does not compensate for the expense 
and the trouble which the yearly course at such places entails, 
and that it is wiser to remain in his comfortable home or to 
betake himself into the country and enjoy the mountain or for- 
est air in some place where he can live in peace. The life in 
modern watering-places is not adapted for a patient with mye- 
litis. Mild cold-water treatment in an intelligently conducted 
sanitarium (Graefenberg, Nassau, Elgersburg, and others) may 
well be recommended. All internal medicines (strychnine, 
silver, ergotine, iodide of potassium, etc.) are of no avail. The 
treatment of the retention of the urine and the consequent 
cystitis must be carried out according to strict surgical princi- 
ples. In the treatment of a compression myelitis we must not 
forget the necessary extension apparatus, braces, etc., for the 
vertebral column. These means, however, belong to the do- 
main of orthopaedic surgery. 

Sometimes the effect of a unilateral section of the spinal 
cord, where we consequently again have a lesion of the gray as 
well as the white matter, can be observed in those rare in- 
stances in which traumatism, a tumor, or the like, has rendered 
the half of the cord incapable of performing its functions. The 
clinical picture resulting from such a lesion is much more rarely 
observed than w r e should be led to suppose from the accounts 
in the text-books. The disease is called Brown-Sequard's spi- 
nal paralysis. It, in short, manifests itself as a motor paralysis 
on the side of the lesion, and a sensory paralysis on the oppo- 
site side. This is explained by the distribution of the fibres, 
inasmuch as the sensory fibres cross over to the other side soon 
after their entrance into the cord, while the motor fibres pass 
upward to the medulla oblongata without crossing (cf. Fig. 
147) ; thus, if, for instance, the lesion be in the right half of the 
lumbar cord, a paresis of the right leg ensues, while the left is 
anaesthetic; if the lesion is high up in the right half of the 



BR WN- SEQUARD'S PA RA L YSIS. 



457 



cervical cord, the right arm and right leg are paralyzed (" spi- 
nal hemiplegia "), and the other half of the body is anaesthetic. 
The fact that on the side on which there is motor paralysis 
there is often a hyperaesthesia (Kiver has reported a case in the 
Neurol. Centralbl., i8qi, 



« 



t 




A\ 




No. 2, in which there was 
no hyperesthesia) for cer- 
tain qualities of sensation 
— with the exception of 
the muscular sense, which 
appears diminished — is 
explained, according to 
Brown - Sequard, by the 
fact that the fibres for the 
muscle sensibility do not 
cross over as the other 
sensory fibres. Above the 
hyperaesthetic there is an 
anaesthetic zone, due to 
the destruction of the pos- 
terior nerve roots. Fur- 
ther, there is an increase 
of the reflexes on the side 
affected with motor paral- 
.ysis, owing to the cutting 
off of the inhibitory influ- 
ence, as well as a vasomo- 
tor paralysis, manifesting itself by an elevation of tempera- 
ture. On the anaesthetic side the reflexes are normal ; a narrow 
hyperaesthetic zone (on the trunk) is here also noticeable above 
the area of anaesthesia. 

On the whole, the descriptions which we possess of uni- 
lateral cord lesions are of no great practical use, because, as 
has been stated, the clinical picture just described is but rarely 
distinct and complete, and may present all kinds of variations 
(cf. Hoffmann, Deutsch. Arch. f. klin. Med., 1886, 38, 6, where 
three cases of this class which occurred in Erb's clinic are 
described). 

LITERATURE. 

Peabody. New York Medical Record, February 5, 1883, xxiii. 

Charpentier. Revue d'hyg., March 3, 1883, v. 

Barlow. Lancet, November 20, 1886, ii. (Myelitis after Measles.) 



Fig. 147.— Schema of the Course of the 
Nerve Fibres in the Spinal Cord, v, un- 
crossed motor fibres, v ', uncrossed vaso-motor 
fibres, sm, uncrossed fibres for the muscular 
sense, s, decussating sensory fibres. (After 
Brown-Sequard. ) 



4 58 DISEASES OF SUBSTANCE OF SPINAL CORD. 

Grasset et Estor. Myelite cervicale. Revue de med., 1887, vii, 2. 

Schutz. Prager med. Wochenschr., 1887, xii, 38. (Cure of Myelitis.) 

Cramer. Arch. f. Psych, u. Nervenkrankh., 1888, xix, 3, p. 667. 

Kroger. Beitrage zur Pathologie des Riickenmarkes. (Recovery from Com- 
pression Paralysis.) Dorpat, 1888. Inaug.-Dissert. 

Gessner. Arch. f. Augenheilk., 1888, xix, 1. (Myelitis Acuta after Loss of Blood.) 

Herter, A. Christian. A Study of Experimental Myelitis. Journ. of Nerv. and 
Ment. Diseases, 1889, xiv. 

Schmaus. Die Compressionsmyelitis bei Caries der Wirbelsaule. Wiesbaden, 
Bergmann, 1889. 

Schaffer. Neurol. Centralbl., 1891, 8. 

Oppenheim. Zum Kapitel der Myelitis. Berliner klin. Wochenschr., 1891, 31. 

Rosenbach, P. und Schtscherback. Ueber die Gewebsveranderungen des 
Riickenmarks in Folge von Compression. Virch. Arch., lxxii. 

Eulenburg. Spinale Halbseitenlasion mit cervico-dorsalem Typus nach In- 
fluenza. Deutsche med. Wochenschr., 1892, 38. 

Leyden. Zeitschr. f. klin. Med., 1892, xxi, 1, 2, 5, 6. 

II. Spinal Lesions regarded from their Pathological 
Aspect — Pathological Diagnosis. 

i. affections of the spinal cord due to diseases of 
the blood-vessels. 

A. Diseases of the Arteries of the Spinal Cord and their Consequences. 

The vertebral arteries which arise from the subclavian, and which 
unite to form the single basilar artery, give off, after having entered 
the skull, an anterior spinal and a posterior spinal artery by which 
the spinal cord is supplied with blood. The anterior spinal arteries 
of both sides unite to form a vessel which runs along the spinal cord 
in the anterior spinal fissure, while the posterior spinal arteries anas- 
tomose freely with each other without, however, completely uniting; 
the horizontal branches run along the septa. White and gray matter 
are nourished in the same way, but the capillary network of the 
latter is much denser than that of the white substance. 

The venous blood is collected into two fairly large veins, which are 
called the central veins of the spinal cord. They anastomose freely 
among themselves, and are connected with the anterior and posterior 
spinal veins. From them the venous blood passes into the vertebrals, 
which empty into the innominate or the subclavian vein. About the 
diseases of the spinal veins up to the present nothing is known. 

/. Spinal Hemorrhage — Hmnorrhagia {or Apoplexia) Mednllce 
Spinalis — Hcematomyelia. 

While, as we have shown above, a primary haemorrhage 
from the cerebral vessels is one of the most common causes 



HJS.MA TOM YE LI A . 



459 



of lesions of the brain, spontaneous haemorrhages from the 
spinal arteries are exceedingly rare, and indeed it seems hardly 
possible that a haemorrhage could take place into the sub- 
stance of the cord, so firmly held together as it is by the tough 
pia mater, without the previous existence of alterations in its 
consistence ; besides, the anatomical conditions of the arteries 
are such that the blood pressure is decidedly lowered before 
the blood wave reaches the spinal cord ; furthermore — and this 
is perhaps the most important reason for the rare occurrence 
of haemorrhage into the cord — miliary aneurisms, which in 
the brain are the most frequent source of haemorrhage, are 
never found here. For these reasons the existence of primary 
spontaneous spinal haemorrhages has been absolutely denied, 
and it has been assumed that in every case changes in the con- 
sistence of the cord substance must have preceded. We fully 
agree with those who believe in their extreme rarity, but, 
nevertheless, we are of the opinion that under certain condi- 
tions primary haemorrhages actually do occur. Such condi- 
tions are: (i) in old persons the coexistence of cerebral haem- 
orrhages in consequence of arterial disease ; (2) the presence 
of such aetiological factors as excessive muscular exertion 
(heavy lifting, cutting wood, etc.) ; (3) the sudden and violent 
suppression of haemorrhages in other places (the menses, haem- 
orrhoids, etc.) ; (4) the exposure to a sudden marked diminu- 
tion of atmospheric pressure, as happens to those who follow 
certain occupations, as, for instance, workers in compressed 
air in building bridges or winning amber (cf. Hirt, Gewerbe- 
krankheiten im Handbuch der spec. Pathologie und Therapie, 
vol. i, third edition, reprint, pp. 83 et seq.). 

The pathological condition is either one of capillar}' haem- 
orrhages or of a haemorrhagic infiltration in which the escaped 
blood extends between the nerve fibres along the course of the 
vessels, or finally we have haemorrhagic foci, in which the blood 
coming from the vessels in larger quantities presses the nerve 
tissue apart and forms a sort of cavity. The focus usually ex- 
tends in the longitudinal direction of the cord. Haemorrhage 
may occur at any level of the spinal cord and in any portion of 
the cross-section, and may produce the same changes in its sub- 
stance as cerebral haemorrhage produces in the brain — changes 
with which we have become familiar in a previous chapter. 

Clinically, spinal apoplexy is characterized by paralysis 
with a sudden onset, sometimes attacking the patient without 



460 DISEASES OF SUBSTANCE OF SPINAL CORD. 

any premonition and while he is apparently in the best of 
health ; he suddenly sinks to the ground without losing con- 
sciousness, and is deprived of the use of his limbs ; occasion- 
ally prodromata, such as tearing pains or formication in the 
limbs, may precede for hours or days. The extent and the 
degree of the paralysis depend entirely on the seat of the 
haemorrhage ; it may be confined to one half of the body, or to 
both legs or to both arms, or it may take in all four extremities 
simultaneously. It develops extremely rapidly, and reaches its 
fullest extent within twenty-four hours. If this is not the case 
it is not a spinal haemorrhage with which we are dealing. Pains 
and rigidity of the back and clonic muscular twitchings are 
equally constant, as are the bladder symptoms, which are prob- 
ably never absent in hsematomyelia. With regard to sensation 
and the reflexes no general rule can be given, yet an increase 
of the reflexes immediately after the catastrophe is not exactly 
rare. Death may occur within a few hours, an event which is 
especially likely to take place if the haemorrhage is situated 
high up. In other cases the patient lives for days and weeks, 
and dies from the effects of bed-sores, of a cystitis, etc. Finally, 
at least relative recovery is not excluded ; the patient may either 
get over the effects of the lesion, or he may be left with motor 
or sensory disturbances of the most varied kinds. The differ- 
ential diagnosis between hsematomyelia and hsematorrhachis 
(meningeal apoplexy) has been discussed above. For the treat- 
ment we may try the application of ice to the spinal column 
and the internal administration of ergotine. The success of 
these measures is always very doubtful, and a careful attention 
to the nutrition and the cleanliness of the patient should in all 
cases be considered the thing of most importance. 

2. Embolism and Thrombosis of the Spinal Arteries and 
Myelomalacia. 

Embolism of the spinal cord, the development of which has 
been studied experimentally by Panum, is extremely rare in 
man, probably owing to the smallness of the spinal arteries and 
the fact that they arise at right angles. The symptoms by 
which emboli manifest themselves are not definitely known ; 
possibly there is a connection between embolic processes and 
the so-called choreic movements, but this is still hypothetical. 

It is about the same with arterial thrombosis, the independ- 
ent existence of which is, to say the least, doubtful, but since, 



EXD ARTERITIS SP IX A IIS. 46 r 

as Leyden has pointed out (Ruckenmarkskrankheiten, ii, 41), 
disease of the spinal vessels is extremely common, the occur- 
rence of arterial thrombosis is very easily possible. Not only 
the inflammatory processes in the spinal cord, which are ac- 
companied by arterial disease, but also the senile changes, 
which consist in fatty degeneration and thickening of the ves- 
sel walls, predispose to it. The necrosis which occurs in the 
substance of the spinal cord in consequence of arterial obstruc- 
tion is similar to that described on page 244 as occurring in 
the brain substance. The condition of softening is called mye- 
lomalacia (cf. also Redlich, Ueber eine eigenthlimliche, durch 
Gefassdegeneration hervorgerufene Erkrankung der Riicken- 
markshinterstr'ange, Prager Zeitschr. f. Heilkunde, 1.89 1, xii). 

j. Endarteritis {syphilitica). 

That the spinal arteries participate in the process which 
Heubner has shown to occur in the cerebral arteries (page 
252), according to competent observers, does not seem to ad- 
mit of doubt. It is equally certain that this process plays here 
a relatively smaller role than in the brain. Heubner himself, 
Knapp, Leyden, and others have reported interesting observa- 
tions bearing on this, and it seems that an endarteritis oblit- 
erans in the spinal cord leads either to a myelitis or a multiple 
sclerosis. Rumpf, in his excellent treatise on The Syphilitic 
Diseases of the Nervous System (page 349), has published in 
full a very interesting case of syphilitic disease of the spinal 
arteries, which was followed by a similar report bv Knapp 
(Neurol. Centralblatt, 1885, 21), and another by Graeff (x\rch. 
f. Psych, und Nervenkr., 1882, xii, 3). There are, however, 
only comparatively few cases to be found in the literature, and, 
in almost all, syphilis of the brain coexisted with syphilis of 
the spinal cord, and endarteritis obliterans was almost alwavs 
demonstrable in the brain as well. Two interesting cases 
have been reported by Schmaus (Deutsch. x\rch. f. klin. Med., 
1889, vol, xliv, 2, 3, p. 244). In one of them the syphilitic af- 
fection took the form of an arterial disease, running a subacute 
course with hyaline fibrous thickening of the intima and simul- 
taneous inflammatory infiltration of the whole vessel wall, 
which was followed by an irregular disseminated patchy scle- 
rosis of the white matter, a marginal sclerosis, and a degenera- 
tion of Goll's columns in the cervical cord. That the degen- 
eration of the nerve parenchvma was attributable to the low 



462 DISEASES OF SUBSTANCE OF SPINAL CORD. 

state of nutrition in consequence of diminution in the blood 
supply seemed beyond doubt. As for the symptoms, sensory 
disturbances (pains, parassthesias, hyperaesthetic zones) and 
motor disturbances (at first fatigue and finally complete para- 
plegia), furthermore incontinence of the urine and fasces, con- 
stituted the clinical picture. In the second case a syphilitic 
degeneration of the vessel walls combined with a poliomyelitis 
was found. With our present knowledge we must content 
ourselves with diagnosticating a diffuse affection of the spinal 
cord, a transverse myelitis, a tumor, and the like. The diag- 
nosis of a syphilitic disease of the arteries must be made with 
reservation during life, and must only be assumed when the 
luetic history is certain. 

LITERATURE. 

Ewald. Ueber syphilitische Gefasserkrankung. Berl. klin. Wochenschr., 1889,48. 
Reynolds. Syphilitic Disease of the Spinal Cord. Brit. Med. Journ., 1889, p. 1221. 
Siemerling. Arch. f. Psych., 1890, xxii, 1. 
M oiler. Studier ofver ryggmargssyfilis. Nordisk. med. Ark., 1890, xxii, 4, No. 22. 

/f.. Dilatation of the Spinal Arteries. 

We know very little about aneurisms of the spinal arteries. 
Besides the case reported by Liouville, which is also quoted by 
Leyden (loc. cit., 2, p. 42), none can be found in the literature. 
The question, therefore, whether syphilis may give rise to 
aneurisms here can not be answered. It is possible that bodily 
exertion has a predisposing action. A symptomatology and a 
therapy do not exist for aneurisms of the spinal cord. (Spen- 
cer, Sequel of a Case of Traumatic Aneurism of the Spine, 
Brit. Med. Journ., 1891, December 5.) 

5. Neuroses of the Spinal Arteries. 

The vaso-motor nerves of the spinal arteries behave just 
like those that supply the cerebral vessels, and upon whether 
they are in a state of irritation or in one of paralysis the amount 
of blood in the spinal cord depends. But easy as it is to dem- 
onstrate hyperaemia and anaemia of the cord in the cadaver, it 
is difficult, on the other hand, to say in what way changes in 
the amount of blood in the spinal cord influence the health of 
the patient, and whether a greater or lesser fullness of the ves- 
sels, or frequent fluctuations between the two, are attended 
with any marked symptoms. All views on this subject are 
entirely hypothetical. The pathological changes in the spinal 



NEUROSES OF THE SPINAL ARTERIES. 



463 



cord, due to an artificial transient anaemia produced by liga- 
tion of the abdominal aorta, Spronck (Arch, de physiol. norm, 
et pathol., September 1, 1888, xx), following out the early re- 
searches of Brieger and Ehrlich, has lately demonstrated, with- 
out, however, throwing any further light upon the clinical 
symptoms caused by spinal anaemia. 

Since the time of Peter Franck ( 1 79 1 ) there has been a wide- 
spread opinion that hyperaemia of the spinal cord can give 
rise to a number of symptoms of irritation, some of which 
being motor, some sensory, together make up the clinical pic- 
ture of what has been described as spinal irritation. But the 
fact that it was found impossible to accurately define a clinical 
picture indicative of this condition and the difficulties which 
arose in the diagnosis have led most observers to abandon the 
term. The disease used to be described somewhat as follows : 
The patients, who, as a rule, are females belonging to the best 
classes of society, complain of an occasional feeling of fatigue 
and of pains in the back, which are intensified by the erect pos- 
ture. Walking becomes difficult, and the gait is that of an old 
person ; they walk with a bent back and take each step with 
care. Painful sensations, paraesthesias, formication, and numb- 
ness in the lower extremities are complained of. The functions 
of the bladder are more or less disturbed ; often there exists a 
uterine catarrh. The patient is low-spirited, and has a tend- 
ency to hypochondriacal notions. On examination, we find the 
reflexes either normal or exaggerated ; sensibility is somewhat 
affected, and disseminated anaesthetic plaques are demonstra- 
ble. A certain tenderness over the vertebrae is almost always 
noted ; it is usually more pronounced in the lumbar and dorsal 
than in the cervical region. The course of the disease is emi- 
nently chronic ; often months and years pass, notwithstanding 
all therapeutic measures, before any decided improvement oc- 
curs, and those unfavorable cases in which the patient finally 
becomes bed-ridden and, after having been for years affected 
with paresis or paralysis, falls at last a prey.to an intercurrent 
malady, are by no means exceptional. A cause was often looked 
for in vain. Overexertion or sexual excesses were regarded as 
sometimes indirectly giving rise to the disease ; at times the 
immoderate indulgence in tobacco was blamed, but more fre- 
quently all such factors were wanting, and a congenital weak- 
ness of the nervous system had to be made responsible for the 
affection. 



464 DISEASES OF SUBSTANCE OF SPINAL CORD. 

Further investigations must teach us to what extent the 
affection described by Mobius under the term akinesia algera 
is qualified to replace " spinal irritation." Certainly only the 
severest form of the latter could be represented by this condi- 
tion, which, according - to Mobius, is characterized by severe 
pain on the slightest exertion, so that there exists a total inabil- 
ity to move. The observations of Mobius have been confirmed 
by many others, but it is not yet clear whether the condition 
represents a separate disease or not. 

The treatment in this condition, just as was the case for 
spinal irritation, should be local and general. The former con- 
sists in the early and energetic use of the Paquelin cautery and 
of the constant current (descending) ; the latter in the use of 
tepid baths and tonics. Yet often all measures are fruitless, and 
it is advisable to be very guarded in giving an opinion with 
regard to the duration and probable outcome of the disease. 

LITERATURE. 

Mobius. Akinesia algera. Deutsche Zeitschr. f. Nervenhk., 1891, i, 1,2. 

Kbnig. Centralbl. f. Nervenhk., 1892, xi, p. 97. 

Mobius. Deutsche Zeitschr. f. Nervenhk., 1892, ii, 5. 

Longard. Ibid., 1892, ii, 5. 

Erb. Ibid., 1893, iii, 1-3. 

Putnam. Boston Med. and Surg. Journ., 1892, cxxvii, 10. 

Moyer. Med. Standard, Chicago, January, 1893, xiii, 1. 

That a chronic anaemia of the substance of the spinal cord 
may give rise to a paralysis, especially of the lower extremi- 
ties, which may last for years, seems probable according to the 
thesis of Meunier (Paris, 1886), yet nothing certain can be said, 
especially as in the cases in question it may be difficult to ex- 
clude hysteria. 

Just to what class we must assign those instances of paraly- 
sis, described more especially by Russell Reynolds, which de- 
pend on the imagination — whether they are due to functional 
disturbances in the spinal cord, or whether, under the influence 
of psychical activity in consequence of auto-suggestion, a dis- 
ease of the whole nervous system develops — is not known. 

The various disturbances in the sexual functions — for in- 
stance, the impotentia coeundi, which is quite a common mani- 
festation of a functional disturbance of the spinal cord in young 
and middle-aged men — we shall enlarge upon in the chapter 
on neurasthenia. 



ACUTE MYELITIS. 



465 



II. INFLAMMATORY PROCESSES IN THE SUBSTANCE OF THE 

SPINAL CORD. 

/. Purulent Myelitis — A bscess of the Spinal Cord. 

While circumscribed pus formations in the brain substance 
are by no means rare, the formation of an encapsulated pus 
focus in the spinal cord is one of the greatest exceptions. Al- 
though Leyden succeeded in producing such foci experimen- 
tally in dogs, the clinical observations in man are so few that it 
is impossible to formulate from them a definite symptoma- 
tology. Pathologically, it is interesting to note that Ollivier 
and Jaccoud (quoted by Leyden, loc. eit., ii, 205) have seen ab- 
scesses which varied in size from that of a bean to that of a 
hazel-nut and were filled with a greenish-white pus. They 
were situated some in the cervical, some in the dorsal cord. 
The symptoms, on the whole, were those of a grave, acute soft- 
ening. In an article by Ullmann (Zeitschr. f. klin. Med., 1889, 
xvi, 2, page 39) an interesting discussion on spinal abscesses 
and an exhaustive collection of references will be found. 

2. The Non-purulent Myelitis. 
Inflammatory processes in the spinal cord are very fre- 
quent. In the majority of cases they are of a chronic type 
and less often acute. With reference to their situation, we have 
already stated that they may implicate the white as well as the 

gray matter. 

A. The Acute Form. 

As we said on page 449, we have in acute myelitis a process 
which is characterized by the death of the nerve elements and 
a secondary increase of the connective tissue. In the acute 
stage a change in the consistence of the cord takes place ; the 
parts become softened and appear swollen and infiltrated. Sec- 
tions of the cord are not so distinct, and the demarcation be- 
tween the white and gray matter is less sharp. The color may 
be reddish (hemorrhagic), yellowish-red, rusty brown, whitish, 
or of any intermediate shade. The extent of the process of 
softening varies. It may be spread over the whole or only a 
part of the cross-section, and may extend longitudinally for a 
greater or less distance. Sometimes disseminated foci are 
found not only in the cord, but can also be demonstrated in the 
brain. We shall speak about these later. 

In exceptional cases, which are difficult to explain, abso- 
30 



466 DISEASES OF SUBSTANCE OF SPINAL CORD. 

lutely no changes were found at the autopsy, although the 
course of the disease seemed in every way to suggest an acute 
lesion. These patients were for the most part young, and up 
to the time of their illness vigorous persons. After a short 
prodromal stage, in which there were headache and some fever, 
they were attacked by a flaccid paralysis of both legs, which 
developed in a few days. To this was added in a very short 
while paresis of both arms, so that the helplessness of the pa- 
tients reached an unusual degree. The condition of the reflexes 
and the electrical excitability varied in the few cases reported 
up till now. According to the records, the functions of the 
bladder and rectum as well as sensibility remained normal. 
The prognosis is very doubtful. Sometimes bulbar symptoms 
appear, and the patient dies within from eight to fourteen days 
after the onset. Sometimes the course is more protracted and 
some improvement occurs, which, however, is never complete. 
The affection which presents the clinical picture just described is 
called Landry's paralysis {i^g), paralysie ascendante aigue, acute 
ascending spinal paralysis, although it is not definitely known 
whether we actually have to deal with a spinal affection and 
not rather with a very acute infectious peripheral neuritis. 
Until we possess the results of a larger number of anatomical 
examinations it is of no use to theorize any more about the 
nature of the disease (cf. Schultze, Schwarz, Bernhardt, von 
Recklinghausen, and Klebs (who found hyaline thromboses), 
Munch, med. Wochenschr., 1890, 52, pp. 923 et seq.). 

With regard to the aetiology of Landry's paralysis, about 
which so little is known, it is possible that it may be caused by 
infectious diseases, for instance, by whooping-cough (Mobius). 
Of great interest is the communication of Curschmann (Ver- 
handl. des flinften Congresses fur innere Med., Wiesbaden, 
1886, p. 469), in which he speaks of a case of acute ascending 
paralysis where at the autopsy typhoid bacilli were found in 
the spinal cord. It may also develop in the course of perni- 
cious anaemia (cf. also Minnich, Zeitschr. f. klin. Med., 1892, 
xxi, 1, 2). 

The symptomatology, diagnosis, and treatment of acute 
myelitis have been discussed on pages 450 to 456. 

LITERATURE. 

Schultze. Berliner klin. Wochenschr., 1883, 39. 

Hoffmann. Arch. f. Psych, und Nervenkrankh., 1884, xv, 1, 140. 



SPINAL TUMORS. 467 

Bernhardt. Zeitschr. f. klin. Med., 1886, p. 391. 

Pitres et Vaillard. Arch, de physiol. norm, et pathol., fevr. 1887, p. 149. 

Dixon Mann. British Med. Joum., March 26, 1887. 

Iwanow. Zwei Falle von acuter aufsteigender Spinalparal. Petersb. med. 

Wochenschr., 1888, 46. 
Schwarz. Zeitschr. f. klin. Med., 1888, xiv, 3, p. 293. 
Woodward. British Med. Joum., November 3, 1888. 
Neuwerck und Barth. Zur pathol. Anatomie der Landry'schen Lahmung. 

Ziegler's Beitrage zur pathol. Anat. und allgem. Pathol., 1889, Heft 1. 
Behmer. Die Landry'sche Paralyse. Inaug.-Dissert., Berlin, 1890. 
Leyden. Ueber gonorrhoische Myelitis. Zeitschr. fur klin. Med., 1892, xxi, 5,6, 

p. 607. 
Eisenlohr. Ueber primare Atrophie der Magen- und Darmschleimhaut und 

deren Beziehung zu schwerer Anamie- und Riickenmarkserkrankung. 

Deutsche med. Wochenschr., 1892. 49. 
Hlava. Poliomyelitis acuta disseminata (Paralysis Landry). Arch, bohemes de 

med., 1 891, t. iv, Fasc. 2. 
Albu. Zur Aetiologie der Paral. asc. acuta. Zeitschr. f. klin. Med., 1893, xxiii, 

5,6. 
Leyden. Multiple Neuritis und aufsteigende acute Paralyse nach Influenza. 

Zeitschr. f. klin. Med., 1894, xxiv, 1, 2. 
Jolly. Ueber acute aufsteigende Paralyse. Berliner klin. Wochenschr., 1894, 12. 

B. The Chronic Form. 
Chronic myelitis is much more commonly observed than 
the acute form. It is characterized by the death of the nerve 
elements and a consequent increase of the supporting elements, 
which gives to the tissue a peculiar firm appearance and con- 
sistence — sclerosis. That this sclerosis is frequently confined 
to certain nerve tracts, giving rise to the so-called " system-dis- 
eases," we have pointed out above on page 440. On page 451 
will be found some account of the sensory, motor, and trophic 
changes which are found in these affections. It is in all cases 
of great importance to look to the condition of the reflexes, as 
this may have a decisive significance for the diagnosis. The 
disturbances of the bladder and rectum in chronic myelitis and 
the treatment of the disease have been discussed above. 

III. Spinal Tumors. 
Pathological Anatomy. — In the spinal cord, just as in the 
brain, the glioma is relatively the most frequent form of pri- 
mary neoplasm. What has been said on page 289 about its 
development holds good here also. The cervical and dorsal 
part of the cord seem by preference to be the seat of the gli- 
oma. Sarcomata, which from the onset present a sarcomatous 
nature, and gliosarcomata — that is, gliomata with unusually 



468 DISEASES OF SUBSTANCE OF SPINAL CORD. 

marked proliferation of cells — have been observed, although but 
rarely as primary tumors. Angiomata, small reddish, probably 
congenital (Virchow) foci, have been found, and Ganguillet has 
observed a cylindroma in the lowest portion of the spinal cord. 
Solitary tubercles and syphilomata are much rarer here than 
in the brain. Carcinomata usually start from the vertebrae and 
afterward spread to the spinal meninges. The secondary 
changes are, of course, not nearly so well marked here as those 
found in the brain, since the spinal cord is in a position to offer 
greater resistance to the growth that presses upon it. Only 
when the tumor has reached some considerable size — e. g., that 
of a hazel-nut — do symptoms analogous to the so-called " indi- 
rect symptoms " in the brain make their appearance. 

etiology. — The aetiology is absolutely unknown. Though 
in certain cases traumatism has been made responsible for 
gliomata in the spinal cord, we are still in complete igno- 
rance about the real cause, as we confessed ourselves to 
be when treating of their occurrence in the brain. The in- 
fluence of age and sex here is the same as in tumors of the 
brain. 

Symptoms. — If a patient complains of persistent pains and 
stiffness in his back, if at the same time there are found sensory 
disturbances in the form of paraesthesias, circumscribed areas 
of anaesthesia, and motor disturbances in the form of slowly 
but steadily progressing paralysis of one or more extremities, 
the suspicion that a tumor of the meninges or of the cord itself 
exists, is justifiable. The likelihood is greater if other spinal 
affections can be excluded and if occasional remissions in the 
progress of the disease can be noted. It is true the diagnosis 
of spinal tumors always remains a very difficult thing, and at 
times, for instance, we may not be able to definitely differenti- 
ate a myelitis from a spinal tumor. This is easily understood 
if we consider that spinal tumors may give rise to the most 
varied clinical pictures, according to their position and size 
and according to the greater or lesser involvement of the 
white or gray matter. There is no doubt but that a tumor 
of the spinal cord may give rise to symptoms of a compres- 
sion myelitis, of tabes, or of a myelitis, and that if it be con- 
fined to one side it may produce the symptoms of a Brown- 
Sequard paralysis. Roth (cf. lit.) claims that loss of the tem- 
perature sense is frequently observed in spinal glioma, and that 
this, combined with analgesia, paresis, and muscular atrophy, 



SPINAL TUMORS. 



469 



is sufficient to settle the diagnosis. The considerable mate- 
rial which Roth has at his disposal makes his monograph very 
valuable. It is only to be expected that vaso-motor as well as 
trophic symptoms should be found. To interpret these must 
be left to the physician's skill in diagnosis, upon which so much 
depends in the recognition of tumors of the cord. Sudden 
changes in the spinal symptoms, temporary remissions, then 
again sudden changes for the worse, should all be made to 
have their proper diagnostic value. In cases of well-marked 
paraplegia dolorosa, where we have tearing pains in the small 
of the back, radiating into the extremities, together with atro- 
phy of the muscles of the lower legs, we should always think 
of one or several tumors of the cauda equina. In these cases 
contractures of such severity sometimes develop that the heels 
touch the buttocks (Leyden). 

Prognosis. — The prognosis depends upon the nature and 
the seat of the tumor, although the ultimate outcome is always 
unfavorable. If the growth be benign and be situated in a 
relatively indifferent area, the patient may last for years, and 
even enjoy periods so free from discomfort that he may deem 
a recovery quite possible. 

Treatment. — The treatment can only be of any avail if sur- 
gical interference — that is, excision of the tumor — is possible. 
A case of this character has been reported by Gowers and 
Horsley. An oval myxoma which had pressed upon the cord 
was, after removal of the spinous processes of the third, fourth, 
and fifth dorsal vertebras, excised, and the patient recovered 
completely. Bruce and Mott (cf. lit.) diagnosticated intra vitam 
a tumor which, originating in the fifth left dorsal nerve, pressed 
upon the middle of the dorsal part of the spinal cord ; the pa- 
tient presented the symptoms of a compression myelitis and 
died. At the autopsy softening with ascending and descend- 
ing degeneration was found. The authors regret in their paper 
not having decided upon an extirpation of the tumor. 

All other means are fruitless. If there is any suspicion that 
the case is one of syphilis, inunctions with mercury ought to 



be given a trial. 



LITERATURE. 



Lloyd and Deaver. A Case of Tumour of the Cervical Region of the Spine. 

Journ. of Nerv. and Ment. Diseases, 1889, xiv, p. 228. 
Herter. A Contribution to the Pathology of Solitary Tuber-ie of the Spinal 

Cord. Journ. of Nerv. and Ment. Diseases, 189c, xv, p. 631. 



4/o DISEASES OF SUBSTANCE OF SPINAL CORD. 

Laquer. Ueber Compression der Cauda equina. Neurol. Centralbl., 1891, 7. 

(Lymphangioma Cavernosum Outside of the Dura ; Removal.) 
Eulenburg. Beitrag zu den Erkrankungen des Conus medullaris und der 

Cauda equina beim Weibe. Zeitschr. f. klin. Med., 1891, xviii, 5, 6. 
Rehn. Compression der Cauda equina durch ein Lymphangioma cavernosum. 

Operative Heilung. Arch. f. klin. Chir., 1891, xlii, Heft 4, 

APPENDIX. — PARASITES IN THE SPINAL CORD. 

About parasites in the spinal cord we may look in vain for 
information in the text-books, probably because their occur- 
rence is very unusual, and also because, if they are present, 
they may not give rise to any symptoms. But here we ought 
to make at least brief mention of the cysticerci which have 
been found not only in the brain, but also in the spinal cord. 
Leyden devotes only a few words to this subject in his Klinik 
der Riickenmarkskrankheiten (1,445): "Still more rare [than 
the cysticerci in the brain], 'and as yet of no clinical significance 
whatever, are the cysticerci which may develop ... in the 
adnexa of the spinal cord, etc." I have shown in a case which 
came to my notice, and which I have reported (ci. lit), that 
cysticerci of the spinal cord — there were fifteen or twenty in 
the dural sac — may give rise to symptoms simulating those of 
tabes ; some clinical significance has, therefore, to be attrib- 
uted to them. That the symptoms of spinal irritation, which 
are associated with such parasites in the cord, are not to be 
attributed to the increased intraspinal pressure, but that they 
are of a reflex nature, seems beyond doubt. To diagnosticate 
intra vitam the existence of intraspinal parasites is only possible 
in exceptional cases, as, for instance, if the patient is a butcher 
by trade, or if his frequent indulgence in raw meat gives rise 
to the suspicion of cysticerci ; but even in the most favorable 
cases the diagnosis can not claim to be more than conjectural. 

Almost as rarely do we find echinococci in the vertebral 
canal. A case of this nature, however, which is of a great 
deal of interest, has been published by Jaenicke (cf. lit.). An 
echinococcus, which had existed in the subpleural tissue in the 
region between the ninth and the twelfth dorsal vertebra, pene- 
trated into the vertebral canal, and, owing to the compression 
thus exerted upon the spinal cord, gave rise to such character- 
istic symptoms that the diagnosis intra vitam was to a certain 
degree justifiable. More recently Friedeberg has reported a 
case of this kind in the Centralbl. f. klin. Med., 1893, xiv, 51. 



S YRINGOM YE LI A . 



471 



IV. Congenital Diseases — Hydrorrhachis — Spina Bifida. 

To a collection of fluid in the skull we have given the name 
hydrocephalus (page 308) ; similarly a like collection in the 
vertebral canal we call hydrorrhachis, and specify two forms of 
the disease — the hydrorrhachis externa and interna — accord- 
ing as the fluid is situated in the meshes of the pia or between 
the meninges, or, on the other hand, in the interior of the 
spinal cord. In the latter case we find a dilatation of the cen- 
tral canal, which is either uniform throughout or beaded. 

At the autopsy we not rarely, instead of the normal central 
canal, the ordinary diameter of which measures from one 
tenth to one millimetre, find a canal with a diameter of two, 
five, or even ten millimetres (" hydromyelia "), or alongside of 
the usual canal abnormal cavity formations (" syringomyelia ") ; 
during life, on the other hand, such conditions are by no means 
often correctly recognized. The practical significance of these 
abnormities is not great, as, for one thing, the signs during life 
are so uncertain and changeable that a correct diagnosis has 
almost to be regarded as accidental, and, secondly, because the 
disease, even if recognized, is not at all accessible to any treat- 
ment. Notwithstanding this, it is of course desirable that the 
present state of our knowledge of hydromyelia and syringo- 
myelia should be given briefly here. 

With reference to the origin of hydromyelia, it is more espe- 
cially abnormities in development which we have to deal with, 
and rarely does the influence of pressure — e. g., a tumor in 
the posterior fossa of the skull — come in. For the develop- 
ment of syringomyelia, central gliosis, with secondary disinte- 
gration and cavity formation, is said to play an important part 
(Fr. Schultze). It has recently been doubted that congenital 
developmental anomalies (Leyden, Kahler and Pick, Striimpell, 
and others) are necessary for the occurrence of the alteration. 
Rosenbach and Schtscherback (Virchow's Arch., 1890, cxxii, 
Heft 1) have shown experimentally that cavities may develop 
in compression myelitis as a result of direct or indirect pressure. 
These cavities may connect with the fourth ventricle, and ex- 
tend through the medulla oblongata as far as the conus termi- 
nalis, and in a cross-section two or more lumina may be seen. 
They are of variable lengths, and are, as a rule, situated in 
the lower cervical, in the dorsal cord, and especially in close 
proximity to the central canal, sometimes also in the posterior 



472 DISEASES OF SUBSTANCE OF SPINAL CORD. 

horns. Their width varies from a half to ten millimetres ; their 
contents are sometimes watery and thin, sometimes milky and 
viscid. The relation of the central canal to these cavities varies 
so much that no rule can be given on this point. In certain 
instances it remains intact in its whole length. 

The clinical symptoms which are observed in syringomyelia 
were first described by Morvan in 1883 under the term of 
paresie analgesique a panaris.; hence the condition is some- 
times called Morvan's disease. 

There are, more especially, three symptoms which should 
arouse a suspicion of syringomyelia, namely, (1) localized mus- 
cular atrophies, more especially in the upper extremities ; (2) a 
widespread, non-typical hemianaesthesia (especially analgesia) ; 
and (3) trophic disturbances of the skin and deeper parts (whit- 
low, phlegmon), also of the bones and joints, the former break- 
ing more easily, the latter showing a widening of the capsular 
space, and being covered with villi of varying size and consist- 
ence which are more or less hyperaemic (Ssokolow, Nissen, cf. 
lit.). Extensive neuropathic destructions of bones and joints, 
which occur in consequence of the analgesia, are met with 
(Karg). The muscular atrophy of the upper extremities is 
always associated with more or less pronounced paralysis, as 
we might expect in lesions of the anterior gray horns. In such 
instances amyotrophic lateral sclerosis or peripheral neuritis 
may suggest itself as a diagnosis. The sensory changes are 
readily explained by the fact that the posterior commissure, 
Goll's columns, and the posterior horns are preferably the seat 
of the affection. In one of Schuppel's cases (Arch. d. Heilk., 
1874, xv, p. 44) general anaesthesia was found. It should, how- 
ever, be said that in many instances, instead of anaesthesia, 
hyperaesthesia has been found, which suggested the lancinating 
pains of tabes (Hoffmann, Eisenlohr), and that often all sensory 
changes are absent, so that even these symptoms are far from 
being pathognomonic. The condition of the reflexes varies 
much, as does also the appearance of trophic and vaso-motor 
disturbances under the form of exanthematous eruptions, vesi- 
cles, ulcerations, erysipelatous swellings, etc., which are some- 
times present, sometimes absent. 

From what has been stated, it is obvious that we may meet 
with insurmountable difficulties in attempting to make a diag- 
nosis in cases of syringomyelia, as has been shown, for ex- 
ample, by Charcot in one of his masterly lectures (Arch, de 



SPAY A BIFIDA. 473 

Neurol., 1891, xxii, No. 65). Toxic paralyses, leprous neuritis, 
pachymeningitis cervicalis hypertrophica, trauma of the spinal 
cord, even amyotrophic lateral sclerosis and tabes, may pre- 
sent symptoms which suggest syringomyelia, and the resem- 
blance may be so great that not infrequently the real seat of the 
disease may only be discovered at the autopsy. 

Somewhat related to these dilatations of the central canal 
are those congenital cystic tumors which, penetrating through 
the walls of the vertebral column, make their appearance below 
the skin on the back. If the cyst, the size of which may vary 
from that of a walnut to that of a man's fist, is situated in the 
middle line over the sacrum, it is called a sacro-lumbar myelo- 
meningocele, or spina bifida. The skin over the tumor is either 
normal, or the seat of a hypertrichosis ; the latter is the case in 
spina bifida occulta (Joachimsthal, Berlin, klin. Wochenschr., 
1891, 22; Jones, Brit. Med. Journ., 1891, p. 173; Bartels, Ber- 
liner, klin. Wochenschr., 1892, 33 ; Brunner, Virch. Arch., cxxix, 
p. 246; Joachimsthal, Virch. Arch., 1893, cxxxi, p. 488). Below 
the skin are found the bulging dura and arachnoid. The con- 
tents of the sac, which has sometimes smooth, sometimes rough 
walls, are as clear as water, and identical with the cerebro- 
spinal fluid. The spinal cord is attached to the inner wall of 
the sac by a broad base, or at its point of entrance divides into 
several strands which pass directly into the wall of the cyst. 
The coexistence of a hydromyelus with a spina bifida, the for- 
mer causing an atrophy of the substance of the spinal cord and 
a communication between the central canal and the cavity of 
the spina bifida, is a rarity. 

In a child born with spina bifida we find, as we stated, in 
the middle of the back, in the region of the sacrum, a soft, 
doughy, elastic, not rarely fluctuating tumor, which can be 
made smaller by pressure. The position of the child influ- 
ences the condition of the sac. It is tense in the erect posture ; 
when the child lies down it becomes flaccid and soft, a fact 
which must be referred to the communication usually existing 
between it and the cranial cavity. 

Although the child thus affected may at first develop fairly 
normally, his life is endangered from the first moment. Not 
only does the pressure exerted upon the spinal cord by the in- 
creasing tumor lead to motor and sensory changes, as well as 
bladder symptoms, but there exists a constant menace to life 
which the rupture of the sac would entail, an accident which 



474 



DISEASES OF SUBSTANCE OF SPINAL CORD. 



is favored by the gradual thinning of the overstretched skin. 
Such a rupture is almost always followed immediately by con- 
vulsions and death. 

The aetiology is not known. Possibly we have to do with 
a developmental anomaly, possibly, as Virchow believes, with 
an early formation of partial hygromata (hydromeningo- 
cele). 

The treatment of spina bifida belongs to the domain of the 
surgeon. We may either endeavor to get rid of it by repeated 
puncture and subsequent injections of a solution of iodine in 
glycerine (Morton), or we may content ourselves with method- 
ical compression. Owing to the danger of meningitis, how- 
ever, the whole treatment should always be undertaken with 
great care. 

LITERATURE. 
j. Syringomyelia. 

Kronthal. Zur Pathologie der Hohlenbildung im Riickenmark. Neurol. Cen- 

tralbl., 1889, 20. 
Miura. Virchow's Archiv, 1889, cxvii, 3, p. 435. 
Dejerine. Soc. de Biol, de Paris, Seance du 25 Janvier, 1890. (Changes in the 

Cutaneous Nerves in Syringomyelia.) 
P. Rosenbach und Schtscherback. Zur Casuistik der Syringomyelic. Neurol. 

Centralbl, 1890, 8. 
Bruhl. De la Syringomyelic Paris, 1890. 
Holschewnikoff. Virchow's Archiv, 1890, cxix, Heft 1. (Changes in the 

Peripheral Nerves.) 
Francotte. Arch, de Neurol., 1890, 56-58. 
Joffroy et Achard. Arch, de med. experim., 1890, p. 540. 
Karg. Arch. f. klin. Chir., 1890, xli, Heft 1. 

Hoffmann, J. Syringomyelic Samml. klin. Vortr., N. F., 1891, 20. 
Ssokolow. Wratsch, 1891, 23-25. (Joint Affections in Syringomyelia.) 
Schaffer und Preisz. Hydromyelie und Syringomyelie. Arch. f. Psych, u. 

Nervenkh., 1891, xxiii, 1. 
Bernhardt, M. Deutsche med. Wochenschr., 1891, xvii, 8. 
Charcot. Progres med., 1891, 4. 
Charcot. Arch, de Neurol., 1891, xxii, No. 65. 

Nissen. Arch. f. klin. Chir., 1892, xliv, p. 204. (Joint Affections in Syringo- 
myelia.) 
Koppen. Deutsche Med.-Ztg., 1892, 64, p. 744- 

Oppenheim. Ibid., 1892, 97, p. n 38. (On Typical Forms of Gliosis Spinalis.) 
Schlesinger. Zur Klinik der Syringomyelie. Neurol. Centralbl., 1893, xii, 20.. 
Bernhardt, M. Literar-histor. Beitrag zur Lehre von der Syringomyelie. 

Deutsche med. Wochenschr., 1893, 32. 
Minor. Arch. f. Psych, u. Nervenkh., 1893, xxiv, p. 693. 
Oppenheim. Ibid., 1893, xxv, 2. (A Typical Form of Gliosis Spinalis.) 
Leclerc et Chapuis. Gaz. hebdom. de med. et chir., 1893, 2. ser., xxx, 51. 






SPINA BIFIDA. 



475 



2. Parasites and Spina Bifida, 

Jaenicke. Ein Fall von Echinococcus des Wirbelcanales. Breslauer arztl. 

Zeitschr., 1879, 21, November 7. 
Dollinger. Die osteoplastische Operation der Hydrorrhachis. Wiener med. 

Wochenschr., 1886, xxxvi, 46. 
v. Recklinghausen. Virchow's Archiv, 1886, cv, 2, 3. 
Brunner. Ibid., 1887, cvii, 3. 
Hirt. Ein Fall von Cysticerken im Riickenmarke. Berliner klin. Wochenschr., 

1887, 3. 
v. Recklinghausen. Untersuchungen liber Spina bifida. Virchow's Archiv, 

1887, 105, pp. 243, 275. 
Holt. Remarks upon Spina Bifida. New York Med. Journ., November 5, 

1887. 
Bland Sutton. On Spina Bifida Occulta and its Relation to Ulcus Perforans 

and Pes Varus. Lancet, July 1, 1887, ii. 
Beneke. Fall von unsymmetrischer Diastemato-myelie mit Spina bifida. 

Leipzig, 1888, Festschrift. 
Wichmann. Wiener med. Wochenschr., 1888, 24, p. 837. 
Ribbert. Beitrag zur Spina bifida occulta lumbo-dorsalis. Virchow's Archiv, 

1893, cxxxii, Heft 2. 
Scholl. Fall von Spina bifida occulta mit Hypertrichosis lumbalis. Berliner 

klin. Wochenschr., 1894, 5. 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



In diseases of the general nervous system, the brain and 
spinal cord and the nerves which come off from them all share 
in the morbid process, yet the extent to which the different 
parts are implicated varies very much in different cases. Some- 
times, in so far as clinical symptoms would lead us to con- 
clude, the trouble lies mostly in the brain, sometimes in the 
spinal cord. In the latter case, again, we may have a more 
marked implication of the substance of the cord itself, lesions 
of certain tracts, or perhaps the lesions of the peripheral spinal 
nerves may come more into the foreground. Between such 
extremes there exist manifold intermediate forms, but to say 
much about the course of these diseases which would be appli- 
cable to all becomes all the less possible because a second 
point has to be taken into consideration, namely, whether, and 
if so how far, the whole organism shares in the disease of the 
nervous system. This participation varies in many ways, and 
there are diseases of the general nervous system which can ex- 
ist for years without any serious implication of the general or- 
ganism ; while there are others, and these are far more numer- 
ous, in which sooner or later the nervous disease grows, as it 
were, into a general disease, in which the organs which have to 
do with digestion, circulation, secretion, excretion, sometimes 
even respiration, are affected more or less seriously. That the 
course of the disease and the prognosis must sometimes be 
materially influenced by this we need not say, and one rule is 
forcibly impressed upon us by such cases, a rule which must 
never De lost sight of by the physician, viz., never in a case of 
disease of the general nervous system to content ourselves 
with an examination of the nervous system, but to remember 
476 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



A77 



that the same care must be devoted to all organs without 
exception. This rule, self-evident enough to the conscientious 
observer, we have dared to emphasize again because it is more 
especially in nervous diseases that it has been allowed to fall 
into abeyance. As to the pathology of the diseases which we 
are about to consider, our knowledge is unfortunately in many 
respects very scanty, and in many of them no lesions at all 
have been found after death, although the assumption, that in 
the majority of cases some anatomical changes, macroscopical 
or microscopical, must have been present, would appear to be 
justifiable. Only for certain of the diseases in this category, 
as tabes, dementia paralytica, multiple sclerosis, and certain 
chronic intoxications, have anatomical changes been demon- 
strated, and even here we are not always clear about their 
significance. Again and again it has happened that after an 
accurate analysis of the clinical symptoms a diagnosis has been 
made intra vitam and this and that anatomical change has been 
reckoned upon with certainty, and then at the autopsy the 
whole nervous system was found to be absolutely intact. 
Among such cases we may mention that of Westphal, where 
a multiple sclerosis was diagnosticated ; that of Killian, a sup- 
posed chronic myelitis, a certain case of ophthalmoplegia ex- 
terna progressiva of Eisenlohr and an apoplectic bulbar pa- 
ralysis of Senator (Neurol. Centralbl., 1892, 6). Instead of the 
pathological condition expected, the brain, spinal cord, and 
their nerves were found to be absolutely normal. On the other 
hand, it has happened that where hysteria, epilepsy, or chorea 
had been diagnosticated and one had prophesied most confi- 
dently that the condition of the central nervous system would 
be found normal, the autopsy has shown extensive changes — 
multiple foci in the spinal cord or in the brain cortex, recent 
or old areas of softening, etc. To such errors even the most 
reliable observer is exposed, and it is just the man who has 
observed accurately the greatest number of cases and assisted 
at the post-mortem examination of them who will be most cau- 
tious in his diagnosis and in his prophecies as to what will prob- 
ably be found at the autopsy. 

Uncertain, then, as is the condition of our pathological 
knowledge in these cases, still, if we decide to treat of diseases 
of the general nervous system not simply one after the other, 
but to adopt some arrangement into groups, it is best to base 
this in a general way on the conditions which we find after 



478 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

death, and to distinguish two classes, the first including those 
nervous diseases in which up to the present time no anatomical 
changes have been demonstrated at the autopsy, diseases which 
we therefore call functional neuroses ; the second, those dis- 
eases which are always associated with known anatomical 
changes. 



PART I. 

DISEASES OE THE GENERAL NERVOUS SYSTEM 
WITHOUT ANY RECOGNIZABLE ANATOMICAL 
BASIS. 

" Functional Neuroses." 

In almost all affections which belong- to this group the so- 
called individual predisposition — that is, the personal inherited 
peculiarities — play a prominent role, and in this connection the 
careful studies of Anton (Wien, Holder, 1890) upon the con- 
genital diseases of the nervous system are of undoubted value. 
Nevertheless, it must be said that in many of these cases no 
anatomical changes whatever have been discovered. In pres- 
ence of the number of these conditions it would seem desirable 
to divide them into smaller groups, an undertaking, however, 
that presents the greatest difficulties, because any classification 
must always appear to a certain extent forced. But inasmuch 
as no pathological anatomy enters into the question, it may, 
for practical purposes, be justifiable to group these affections 
according to the influence which the neurosis exercises upon 
the general condition of the patient. It will be found that 
while some of them (though these cases are few) disappear 
after running a shorter or longer course without leaving be- 
hind them anv bad effects, or, even when they last for years, 
never entail serious general symptoms, there are others which 
are characterized not only by their long duration, their obsti- 
nate resistance to treatment, and their tendency to recur, but 
also by the baneful influence which they exert on the general 
system. The former, for the sake of brevity, we shall desig- 
nate as mild, the latter as grave neuroses, although we do not 
mean to exclude the possibility that now and again among the 
ordinarily mild types we may encounter a serious disease run- 
ning a tedious course, while among the grave forms we may 
have cases of far less severity than usual. 

479 



480 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

A further classification might be made according to the 
symptomatology. It is true that the symptoms present so 
many variations that it appears difficult to arrive at any prin- 
ciple according to which we can conveniently group the dis- 
eases, despite the fact that in some cases the symptoms point 
rather to a cerebral, in others more to a spinal affection. Nev- 
ertheless, since we find that in some cases the motor nerves, in 
others the sensory nerves, and again in others the trophic 
nerves, are pre-eminently implicated in the morbid process, we 
may for the present utilize this fact in the arrangement of our 
groups. It is scarcely necessary to state that we are in no 
wise satisfied with this classification, and look upon it only as a 
temporary makeshift, to be superseded as soon as some better 
method shall have been discovered. 



FIRST GROUP. 

NEUROSES WHICH ARE WONT TO RUN THEIR COURSE WITH- 
OUT ANY ESSENTIAL IMPLICATION OF THE GENERAL 
ORGANISM. 

A. AFFECTIONS IN WHICH THE MOTOR NERVES ARE 
CHIEFLY IMPLICATED. 



CHAPTER I. 



CHOREA — CHOREA ST. VITI ST. VITUS S DANCE — BALLISMUS MELAN- 
CHOLIA saltans — Sydenham's disease. 

The term chorea no less than epilepsy is often too loosely 
applied. A person may suffer from chorea-like motor disturb- 
ances without having genuine chorea. Various cerebral and 
spinal affections are capable of producing such symptoms ; but 
a careful observer will rarely find difficulty in deciding whether 
they are the outcome of a functional neurosis or of anatomical 
lesions in the central nervous system. 

By chorea, in the sense in which the term will be used here, 
we mean a functional neurosis characterized by the occurrence 
of peculiar irregular movements entirely beyond the control 
of the patient. They appear in the upper extremities and in 
the face, as well as, though to a lesser extent, in the lower ex- 
tremities and in the trunk. They attack only the voluntary 
muscles, and may persist for days, weeks, and even months 
uninterruptedly, except during sleep. If these movements, as 
is frequently the case, are confined to one side only, to one half 
of the face, to one arm and the corresponding leg, we speak 
of a hemichorea. The distinction which is made in some of 
the older books between chorea major and chorea minor has 
become superfluous, since the symptoms which were formerly 
described as constituting the clinical picture of chorea major 
do not represent an independent disease, but belong to the 
31 481 



482 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

domain of hysteria. Hence we can also dispense with the 
designation " chorea minor." 

The " choreic " movements may appear independently 
where it is impossible to find any coexisting symptoms of 
another disease, or they may be no more than symptoms of 
another affection, be it of the brain or spinal cord. Our exam- 
ination will have to decide between these two possibilities. 
We shall deal here only with the idiopathic, genuine chorea, 
and we need hardly say that only this form is to be regarded 
as a mild neurosis in the sense pointed out above. 

Symptoms. — To describe the choreic movements in detail 
is not easy, because they present very many varieties in de- 
gree and extent. In the relatively severe cases all the muscles 
participate, the head is thrown about and shaken, the neck is 
twisted, the forehead is wrinkled and smoothed, the eyelids 
closed and opened, and the eyeballs rolled around. The facial 
muscles, including those of the lips and the mouth, take part in 
the movements, thus giving rise to the most varied expressions 
— e. g., those of terror, anxiety, or joy — according to the par- 
ticular muscles most strongly affected. Hasse states that the 
tip of the nose may be moved, though I myself have never 
seen this. Very conspicuous are the movements of the tongue 
muscles, since they interfere with speaking, chewing, swallow- 
ing, and with the protrusion of the tongue, which in the worst 
cases become entirely impossible. If the muscles connected 
with the function of respiration are affected, disorders in breath- 
ing are encountered ; the implication of the muscles of the 
trunk gives rise to rotatory and other involuntary movements 
of the body; the patient rises and falls down again, and may 
work himself into the most peculiar and marvelous positions 
("folie des muscles "). 

In the great majority of cases the movements do not 
take place in the way we have described, except, perhaps, 
the twitchings of the face, but are confined to the upper ex- 
tremities, or are at any rate most marked here. Shoulders, 
arms, and fingers are constantly in motion, the affected mus- 
cles twitch, the arms are extended and flexed, the fingers 
spread apart, and so forth. A similar restlessness is observed 
in the muscles of the thigh and calf, the feet are alternately 
lifted, the toes moved, although the lower extremities are gen- 
erally attacked to a lesser extent. Sometimes the movements 
are gone through with lightning quickness, in which rare in- 



SYMPTOMS OF CHOREA. 



43. 



stances the name chorea electrica is justifiable. In milder cases 
the patients may at times be able to remain perfectly quiet, and 
only slight twitchings in the arms, the fingers, perhaps also in 
the facial muscles, will betray the existence of the disease. It 
is a characteristic feature of idiopathic chorea that all move- 
ments entirely cease when the patient is asleep, although going 
to sleep may be rendered somewhat difficult. Once asleep, 
however, such patients rest quietly, and are not disturbed by 
any muscular unrest. 

That the intended movements are influenced by the patho- 
logical ones goes without saying, and it is quite possible that 
at a time when the disease is still at its beginning and has not 
yet been recognized, bat is already exerting its influence upon 
the voluntary movements, the patient may be simply regarded 
as awkward and clumsy. If this happens to children who have 
to write in school, or recruits who have to drill and learn the 
different manipulations, much unpleasantness for the patient 
may arise from this condition, which might have been avoided 
by a careful examination bv a physician. Generally the vol- 
untary movement is normal in its first phase, but soon the 
muscles begin to be seized bv the spasms and the patient is 
not able to carry out the movement intended. This is noticed 
in dressing or eating, or in other ordinary actions of daily life, 
but most of all is it seen in writing, playing the piano, or in the 
performance of other movements requiring a high degree of 
co-ordination, and may even be marked if we ask the patient 
to put out his tongue. 

His apparent awkwardness excites the patient very much, 
and the more he tries to execute the intended movement, the 
more he tries to govern his unmanageable muscles in the usual 
manner, the less he succeeds and the more he is annoyed by 
the involuntarv movements. Only a few particularly well 
disciplined .patients are, at the height of the disease, able to 
keep their muscles for a few moments at absolute rest. The 
reflex and automatic movements are not interfered with. 
Protective movements are performed as by healthy persons ; 
coughing and sneezing are done normally ; neither do the car- 
diac or respiratory movements suffer. 

The sensibility is in no way interfered with. Tenderness 
over the spine may be present, although not regularly. Other- 
wise nothing abnormal can be noticed in the domain of the 
sensory nerves. It is remarkable to note that there is no sense 



484 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

of fatigue, which we certainly should expect after such exces- 
sive muscular action. The body temperature and the urine 
remain normal throughout the disease if no complications are 
superadded. On the other hand, the psychical condition of 
the patients, especially if they be young people, undergoes 
more or less marked changes, which constitute a prominent 
feature of the disease if the course be prolonged ; children who 
have up to this time been kind, obedient, diligent, and willing, 
become willful, peevish, and spiteful; although learning nicely 
and without difficulty and making good progress in school be- 
fore they had any symptoms of chorea, they become slow at 
grasping and understanding what they are taught ; the easiest 
things must be repeated and impressed upon them, and often 
enough they are forgotten again in a few hours. If, and this 
is not rarely the case, an impediment in speech is added in 
consequence of the choreic movements of the tongue, the chil- 
dren become wholly unfit for school. It is at this time no 
longer necessary to advise keeping the child at home, since 
the teachers themselves will no longer permit it to attend. 
The influence of the disease upon the psychical functions is 
generally much less marked in adults. 

In the idiopathic uncomplicated chorea the described mani- 
festations persist usually for several weeks with varying inten- 
sity. From the onset to the cessation of the disease from sixty 
to ninety days may elapse (sixty-nine days, See ; eighty days, 
Jiirgensen ; eighty-nine days, Riecke), yet, as we shall show 
later, the treatment is not without influence upon its duration. 
By far the most frequent issue is recovery, although the possi- 
bility of a relapse is by no means excluded, and in giving a 
prognosis this feature should be taken into account. Death 
from chorea is a very uncommon event, and occurs only in 
very weakly children or when complications arise ; Powell, 
Handford (Brain, 1889), and others have reported fatal cases of 
chorea ; in most instances, however, we are justified in giving 
a good prognosis. 

Complications. — The complications and the relation that 
chorea bears to other diseases deserve much attention, more 
particularly as this relation is to a great extent still obscure. 
In the first place, articular rheumatism must here be men- 
tioned, the connection of which with chorea everybody knows, 
but which, however, is not interpreted by all authors in the 
same manner. While the French writers especially, among 



SYMPTOMS OF CHOREA. 485 

them See and Roger, regard rheumatism as an almost regular 
precursor of chorea, in Germany there is much diversity of 
opinion on this point. Several authorities (Lebert, Eichhorst, 
Strumpell) only state that the two affections are relatively fre- 
quently found together ; others, with Brieger, draw attention 
to the alternating appearance of the two (Berliner klin. Woch- 
cnschrift, 1886, xxiii, 10) ; others, again (Henoch, Litten), look 
upon rheumatism as " the most important and best-founded 
cause of chorea " ; while some, in contradistinction to the rest, 
deny the existence of any connection between the two affec- 
tions (Romberg, v. Niemeyer, Prior). However obscure this 
association may be, to deny it absolutely would be to set facts 
at defiance. According to our own opinion, we have to deal 
with a common noxious agent, an infection which, if chief! v 
localized in the brain, gives rise to choreic movements, while if 
it affects the joints it causes acute rheumatism in them. Most 
probably, we may almost say unquestionably, it is the same 
infectious material which, if affecting the heart, produces endo- 
carditis and myocarditis, for chorea is as frequently connected 
with valvular disease of the heart as with articular rheumatism, 
though the one relationship is as obscure as the other. 

If chorea, or, we had better say, if certain forms of chorea 
are actually to be traced to an infection, we can not be sur- 
prised if choreic movements are found to appear after other 
infectious diseases — e. g., whooping-cough, typhoid fever, diph- 
theria, or cholera. 

The possibility that chorea has some connection with epi- 
lepsy can not a priori be thrown aside. I have twice had occa- 
sion to observe children who up to the age of puberty had re- 
peatedly suffered, as it seemed, from genuine chorea, and who 
afterward became subject to epileptic attacks. It is true the 
tongue was not bitten in these paroxysms, but otherwise all 
the signs of a classical epilepsy were present, not excluding the 
aura. A later communication of Marie (Progr. med., 1886, xiv, 
p. 39), in which the occurrence of ovarian hyperassthesia in the 
course of chorea is mentioned, led us to the idea that possibly 
the above-mentioned attacks were of a hysterical nature, and to 
question whether there may not be certain forms of St. Vitus's 
dance which could be designated as hysterical. 

Lastly, those very rare cases of tropho-neurotic disturb- 
ances in chorea are of interest ; thus, bald spots on the skull 
(Escherich, Mitth. aus der med. Klinik zu Wiirzburg, 1886, ii), 



486 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

or in places absence of pigment in the hair or the skin, were 
noted (Mobius, Schmidt's Jahrb. d. gerichtl. Med., 1886, vol. 
ccix, p. 251). How these are brought about we are utterly un- 
able to explain. 

Diagnosis. — It is usually not difficult to recognize chorea if 
we remember that young patients of the female sex, who are 
often also anaemic, form the largest contingent of the cases, 
that the twitchings chiefly affect the upper extremities and the 
face, and that they are entirely independent of the will of the 
patient. Their disappearance also during^sleep is an important 
point, and this fact by itself would distinguish them from the 
athetoid movements. These latter, possibly the twitchings of 
the tic convulsif, the tremor of paralysis agitans, the shaking 
movements of the intention tremor of multiple sclerosis, finally, 
certain muscular spasms, which Leclerc and Royer (cf. lit.) 
have designated as pseudo-choreas, must more especially be 
taken into consideration, but they ought never to render the 
diagnosis really difficult. 

Pathology. — Our knowledge of the pathology of idiopathic, 
uncomplicated chorea is very imperfect. The changes which 
have been found thus far do not seem to be essential. Repeat- 
edly capillary emboli have been found at the autopsy in the 
thalamus and the corpus striatum, often they could not be dem- 
onstrated (Dana, Brain, 1890, xlix). The experiments of Money 
on guinea-pigs and dogs (Lancet, 1885, 1, p. 985) would indicate 
very decidedly that chorea can be caused by capillary emboli. 
Their mode of action, however, remains unexplained. The ob- 
jections which Litten has raised against the embolic theory, 
that the demonstration of embolic processes in ordinary cases 
of chorea is not proved, and that in spite of the diversity of 
the localization of the foci of softening in the brain the clin- 
ical picture is always the same, can not be regarded as con- 
vincing. 

The communication of Flechsig, who in the two inner ante- 
rior segments of the lenticular nucleus, but nowhere else, found 
small bodies in the lymph sheaths of the vessels, some of which 
were larger, some smaller than blood-corpuscles, has as yet 
been neither confirmed nor overthrown. " Their arrangement 
resembled that of glandular structures; they were strongly re- 
fractive, very firm, and almost like chalk, although they con- 
tained no lime. In alkalies they slowly swelled." Though 
their chemical nature is unknown, they resemble in the main 



/ETIOLOGY OF CHOREA. 48/ 

that material which von Recklinghausen has termed " hya- 
line." This observation has not as yet been interpreted, and 
Flechsig himself declines to give a decided opinion as to 
whether the bodies have been formed in the blood or lymph 
vessels or whether they have to be regarded as products of de- 
generation from ganglionic cells and nerve fibres. Although 
we have to admit that lesions in the lenticular nucleus mav 
cause choreic movements, we can as yet make little use of 
these bodies as an anatomical cause for the disease. Wollen- 
berg regards them as non-essential (Arch. f. Psych., 1891, xxiii, 
1, p. 197). Earlier observations of conditions which were con- 
sidered as significant for chorea — that is, hyperaemia of the 
brain and the spinal cord, lesions of the corpora quadrigemina, 
tubercles in the cerebellar peduncles, inflammatory conditions 
in the vertebras, and spinal irritation resulting therefrom — pos- 
sess only historical interest. 

Although we are then still unable to say anything definite 
about the nature of the disease, the assumption that we have 
before us an affection of the entire nervous system, in which, 
to be sure, the brain takes the most prominent part, seems the 
most probable. Whether certain portions of the brain are par- 
ticularly qualified to produce choreic movements — whether, be- 
sides being produced by irritation of the cortical motor cen- 
tres, they may also be brought about by lesions of the basal 
ganglia ; further, whether this irritation can ever be attributed 
to infectious material, microbes, or the like, whether it can ever 
be connected with fungous growths, such as, for example, Nau- 
nyn has found in the pia belonging to the species of the clado- 
thrix or leptothrix, or whether we have to assume an autoin- 
toxication, as in epilepsy, uraemia, etc. (Duchateau, These de 
Paris, 1893) — all these remain open questions, and we must also 
leave undecided whether or not the alteration of the blood de- 
pending upon the so-called rheumatic diathesis is sufficient for 
the development of the disease. 

^Etiology. — Among the causes of chorea heredity plays an 
important role, as it does in all diseases of the general nervous 
system. This factor is more important, since heredity can here 
not only be called an indirect predisposing circumstance, ow- 
ing to which an individual is more prone to one or the other 
nervous disease, but because there exists actually a hereditary 
form of chorea which is handed down from generation to gen- 
eration and which for a great many years may remain in the 



488 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

family. This chorea hereditaria, or, as it is also called, Hunt- 
ington's chorea, has nothing in common with chorea but the 
name ; it is produced by anatomical changes which have been 
characterized by Oppenheim and Hoppe as a miliary dissemi- 
nated cortical and subcortical encephalitis (Arch. f. Psych., 
1893, xxv, 3). It does not come on in childhood, and hardly 
ever appears before the age of thirty or forty. It is character- 
ized by peculiar motor disturbances resembling those of athe- 
tosis (p. 284), and not rarely leads to pronounced mental deteri- 
oration. It is incurable. The conception that it is a progres- 
sive double athetosis seems to me worthy of consideration (cf. 
Remak, Neurol. CentralbL, 1891, 11, 12; Krohnthal und Kali- 
scher, ibid., 1892, 19; Greppin, Arch. f. Psych., 1892, xxiv, 1; 
and others). There are " chorea families " in which a whole 
generation never remains free from the disease, and only cer- 
tain members are exempt. On the other hand, there exists also 
a chorea congenita (Rau, Inaug.-Dissert., Berlin, 1887), which 
has to be attributed to an affection of the mother caused by 
fright, etc., during pregnancy (Fox, Richter, Mobius, Oppen- 
heim). It has long been known that pregnancy itself may to a 
certain extent predispose to chorea, as is shown by the so-called 
chorea gravidarum. Age and sex play a certain role among 
predisposing causes, inasmuch as the young and the female 
sex are especially prone to it. Among 439 cases, 322 (that is, 
seventy-three per cent) were girls, and 340 (that is, seventy-four 
per cent) were between the ages of five and fifteen ; 41 1 (that is, 
ninety-one per cent) were between the ages of five and twenty 
(Mackenzie). In rare cases old people become subject to chorea 
(chorea senilis), The oldest of my patients was eighty-one, the 
oldest of Mackenzie's patients even eighty-six. 

Among the exciting causes there are two kinds which are 
particularly important — the one, psychical excitement, particu- 
larly fright and anxiety ; the second, frequent contact with in- 
dividuals suffering from chorea, which awakens an impulse to 
imitate the pathological movements and gives rise to what we 
then call chorea imitatoria. The latter is far less important than 
the former. Epidemics of chorea have often been described ; 
Wichmann has observed one in Wildbad (Deutsche med. Wo- 
chenschr., 1890, 30). The time which elapses between the recep- 
tion of the noxious influence and the development of the dis- 
ease usually comprises from five to seven days, sometimes only 
one day. Sometimes, again, the effect follows the cause imme- 



TREATMENT OF CHOREA. 

diately, this being so in ten per cent of all cases caused by 
fright. Besides fright, bodily or mental overexertion, particu- 
larly the latter, may provoke the disease. According to Mac- 
kenzie's report, sixteen per cent of all cases observed are at- 
tributable to this cause. 

Treatment. — Cases of uncomplicated chorea get well with- 
out any interference on the part of the physician, but the re- 
sults of wide and varied experience have taught us that with 
certain measures we are able to cut short the duration of the 
disease to a no inconsiderable extent. With reference to the 
internal treatment it is interesting to follow up the different 
phases and changes through which this has passed in the last 
half century. When the spinal cord was supposed to be the 
seat of the disease much was thought of strychnine, which had 
been recommended by Trousseau and which was administered 
in the form of a sirup. Later, when to the rheumatic basis of 
chorea a prominent place was given, colchicum and quinine 
were preferred. Again, camphor, potassium iodide, and hy- 
drocyanic-acid preparations were prescribed when irritation of 
the sexual organs was held to be the starting point of the dis- 
ease. Venesection, leeches, cups to the head and along the 
vertebral column, were employed for a time on the authority of 
Sydenham. All these measures have now more or less fallen 
into oblivion, and even the zincum oxidum album, once so 
warmly recommended by Hufeland, has had to give way to 
other remedies. Among those still valued, arsenic, which was 
introduced by Romberg, stands first. It is best given in the 
form of Fowler's solution, in doses of from three to five drops 
three times a day, the dose being gradually increased to twenty 
or thirty drops a day. The medicine ought to be well diluted 
with water. Instead of Fowler's solution we might prescribe 
the waters of the Roncegno or Levico springs in doses of a 
teaspoonful to a tablespoonful three times a day. At the same 
time we must be on the lookout for intoxication, which has 
been known to be produced even by small quantities of the 
drug, as was proved by a case of my own. The arsenic treat- 
ment is to be continued until either the symptoms abate or 
digestive disturbances make their appearance, which would 
contra-indicate its continuance. We usually attain our end in 
from fifty to sixty days. 

Next to arsenic we prefer the salicylate of physostigmine 
(eserine), which, in the form recommended by Riess (Berliner 



49<D DISEASES OF THE GENERAL NERVOUS SYSTEM. 

klinischer Wochenschrift, 1887, 22), may be injected hypoder- 
mically twice a day in the dose of one milligramme (y^ gr.). 
Excellent results may be obtained with this mode of treatment, 
and the duration of the disease may be reduced to thirty or 
forty days. We need hardly insist that this drug must be ad- 
ministered most cautiously, because eserine poisoning has been 
observed (Lodderstadt, Berliner klin. Wochenschr., 1888, 17). 
As soon as any bad effects begin to show themselves, such as 
nausea, vomiting, etc., it is advisable to discontinue the medi- 
cine at once for a considerable time. With regard to exalgin, 
so highly spoken of by Dana (Journal of Nervous and Mental 
Diseases, 1892, July), at present I must suspend judgment; 
from small doses I have observed but little effect, while large 
doses did not seem to be always well borne (cf. also Joris, 
Wiener med. Presse, 1892, 44). Antipyrin, which has been rec- 
ommended by Legroux and others, I have completely aban- 
doned. The results obtained with this drug are uncertain and 
transient. We were never able to note cures within from six 
to twenty-seven days with this remedy, such as Legroux has 
reported. If these medicines leave us in the lurch we may with 
caution prescribe chloral, morphine, opium, under the influence 
of which the movements may temporarily abate. 

Among other measures we may mention the use of cold 
water and electricity, which, although only of secondary im- 
portance, may not be without good effects. We have in differ- 
ent places spoken of the cold-water treatment, and wish again 
to repeat here that extremely low temperatures are unneces- 
sary, but that hip baths of 84 F., with cold affusions to the 
back (8i° to 75 F.) and wet packs seem sufficient. In the elec- 
trical treatment the constant current is chiefly to be used, which 
is made to act alternately upon the brain and the spinal cord 
(Hirt, loc. cit., p. 181). 

Sometimes all these means of treatment which we have 
just described are ineffectual. The patients take medicine, 
undergo the cold-water treatment, etc., and no improvement is 
noticeable. In such instances a change of climate is to be 
recommended; the patient maybe advised to travel, and be 
kept away from his family for some time ; excitable individ- 
uals especially, in whom psychical influences increase the motor 
irritation, are to be secluded as much as possible. Visits of 
friends or members of the family should be interdicted. Chil- 
dren should be kept away from school, and should be spared 



TREATMENT OF CHOREA, 



49 1 



any mental exertion. Even at home they should not be made 
to work ; they should be encouraged to suppress the move- 
ments as much as possible, and a small reward should be prom- 
ised if they succeed. In this manner often a good deal is at- 
tained. Only in exceptional cases need the patient be in bed 
for any length of time — namely, if the twitchings are very vio- 
lent and likely to lead to bodily injury. In such instances 
the use of narcotics, as suggested above, becomes more espe- 
cially warrantable. We shall later have occasion to speak of 
the treatment by suggestion ; the results obtained with this 
method are sometimes quite satisfactory. 

LITERATURE. 

Henoch. Berliner klin. Wochenschr., 1883, 52. 

Vassitch. Etude sur les chorees des adultes. These de Paris, 1883. 

Peiper. Chorea bei Typh. abdom. Deutsche med. Wochenschr., 1885, 8. 

Dickinson. On Chorea, with Reference to its Supposed Origin in Embolism. 
Lancet, January 2, 1886. 

Litten. Beitrage zur Aetiologie der Chorea. Charite-Annal., 1886, xi, p. 265. 

Birnbaum. Ueber die Chorea der Erwachsenen. Inaug.-Dissert., Berlin, 
1886. 

Hawkins. Chorea and Epilepsy. Lancet, January 2, 1886. 

Landois. Deutsche med. Wochenschr., 1887,31. 

Mackenzie. Report on Chorea. Brit. Med. Journ., February 26, 1887. (Re- 
ports of the Collective Investigation Committee of the British Medical As- 
sociation.) 

Koch, P. Zur Lehre von der Ch. minor. Deutsches Arch. f. klin. Med., 1887, 
xl, 5, 6. 

Schweinitz. Examination of the Eyes in Fifty Cases of Chorea in Children. 
New York Med. Journ., June 23, 1888. 

Suckling. Brit. Med. Journ., April 28, 1888. (Senile Chorea.) 

Comby. Les relations pathogeniques de la choree. Prog, med., 1888, 16, p. 
300. 

Hoffmann. Ueber Ch. chronica progressiva. Virchow's Archiv, 1888, Bd. iii, 
H. 3. (Huntington's Chorea.) 

Schromann. Deutsche med. Wochenschr., 1888, xiv, 32. 

Mendel. Centraibl. f. Nervenheilk., 1888, xi, 15. 

Lannois. Ch. hereditaire. Revue de med., 1888, 8. 

Chauvreau. Les tics coordines avec emission brusque et involontaire des cris 
et des mots articules. These de Bordeaux, 1888. 

Herringham. Chronic Hereditary Chorea. Brain, 1888, xi, p. 415. 

Jakowenko. Zur Frage der Localisation der Chorea. Centraibl. f. Nervenheilk., 
1888, xi, 22. 

Klippel et Ducellier. Un cas de choree hereditaire de l'adulte. Encephale, 
1888, viii, 6. 

Patella. Contribuzione anatomo-patologica e clinica alia studio della corea mi- 
nore. Padova, 1888. 



492 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



Sinkler. Hereditary Chorea. Boston Medical and Surgical Journ., October 15, 

1888, cxix. 
Sturges. The Relation of Chorea to Rheumatism. Lancet, 1889, i, 3. 
Hegge. Ueber den Zusammenhang zwischen Chorea minor mit der Polyar- 
thritis rheum, und der Endocarditis. Wiener med. Blatter, 1888, 41, 42. 
Schadle. Chorea of the Soft Palate. Phila. Med. and Surg. Rep., October 14, 

1888, lix. 
Gairdner. Case of Nerve Disease with Choreic Movements. Glasgow Medical 

Journ., 1889, xxxi, 1. 
Biernacki. Fall von chronischer hereditarer Chorea. Berliner klin. Wochenschr., 

1890, xxii. 
Remak. Ueber Chorea hereditaria. Neurol. Centralbl., 1891, ii. 
Jolly. Ueber Chorea hereditaria. Ibid. 

Dreves. Ueber Chorea chronica progressiva, Inaug.-Dissert., Gottingen, 1891. 
MacCann. Chorea Gravidarum. Brit. Med. Journ., November 14, 1891, 

p. 1046. 
Lewis. Amer. Journ. Med. Sci., 1892, 3, p. 251. 
Mobius. Ueber Seelenstorungen bei Chorea. Munchener med. Wochenschr., 

1892, 51, 52. 
Guillemet. De la mort dans la choree de Sydenham. These de Paris, 1893. 
Dana. Amer. Journ. Med. Sci., January, 1894. (Microbes as the Cause of 

Chorea.) 



CHAPTER II. 

TETANY TETANILLA TETANUS INTERMITTENS. 

The name tetany (Corvisart) has been given to a neurosis 
which is characterized by paroxysmal tonic muscular spasms, 
during which consciousness remains undisturbed. The spasms 
are often confined to the flexors of the ringers and of the wrist 
joint, and only rarely attack the muscles of the lower extrem- 
ities ; they are always bilateral. The fingers are drawn to- 
gether and the hand assumes, to use Trousseau's comparison, 
the shape which the obstetrician gives it when introducing it 
into the vagina. With these spasms, which are of great inten- 
sity, so that the affected muscles feel tense and hard as boards, 
are associated slight flexion at the elbow joint and a moderate 
adduction of the upper arm. Herard claims that the pressure 
of the thumb upon the other fingers may be so strong as to 
lead to pressure gangrene, but this is unquestionably very rare. 
If the lower extremities are affected the feet assume a position 
of plantar flexion, and the big toe is drawn under the second 
or third. Sensory disturbances are usually entirely absent, 
except that the contracted muscles are painful on pressure and 
the skin over them is covered with a copious sweat. 

These attacks, which vary a good deal in frequency as well 
as in duration, may be produced by pressure upon the larger 
nerve trunks or the larger arteries of the upper extremities, 
as Trousseau found accidentally, by applying a venesection 
bandage; thus, by pressure upon the median nerve or the bra- 
chial artery, a spasm may be produced of exactly the same 
nature as the spontaneous ones. This is called Trousseau's 
sign, and is considered to be of great diagnostic importance. 

The attacks scarcely ever occur suddenly and unexpectedly. 
Generally they are preceded by prodromal symptoms, which 
last for a few minutes and consist in a painful drawing sensa- 
tion of the hands and arms. Previous to the first attack such 

493 



494 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

sensations, together with formication, feelings of coldness, etc., 
may have existed for weeks. The attacks last rarely more 
than five or ten, usually they are over in one or two minutes, 
and it is only in very exceptional instances that they go on for 
several hours. Their frequency also varies, as we have said. 
Some patients — just as now and then happens in epilepsy — 
have not more than one all their life, some have several a day, 
and in others again weeks, months, or years pass between the 
individual attacks, and the disease may extend over twenty or 
thirty years. Jaksch (cf. lit.) distinguishes an acute recurrent 
and a chronic tetany, and thinks that certain forms occur in the 
course of grave cerebral disorders. In all cases, however, pro- 
vided there exist no complications — such as joint affections — 
the outcome is favorable, and in no case can any lasting bad 
effects upon the organism in general be noticed. In the inter- 
vals the patient does not complain of anything and feels in 
perfect health. Only an objective sign is demonstrable, which 
betrays that everything is not going on normally — namely, an 
increase, not only of the electrical, but also of the mechanical 
excitability of the nerves — a condition to which Erb has called 
attention. Even a weak current produces a marked effect, and 
by simply stroking the face with the finger it is possible to 
elicit lively contractions of the muscles supplied by the facial 
nerve. Although this sign is not constant, since it has in cases 
of tetany been looked for in vain, even after the most careful 
examinations, and although we must not forget that it occurs 
not in tetany alone, but also in organic diseases of the spinal 
cord — e. g., in glioma — it remains, nevertheless, very valuable, 
and must certainly be taken into account in the diagnosis. 

The anatomical seat of the disease is still obscure. It has 
been referred to the most varied parts of the nervous system, 
to the cerebrum, the cerebellum, the spinal cord, the periph- 
eral nerves, even to the sympathetic, which seems anyhow to 
be the part of the nervous system which is blamed for affec- 
tions we can not locate. All these, one after the other, have 
been suspected of playing a role in the pathogenesis of tetany, 
but proofs have never been brought forward for the correct- 
ness of any of these views (cf. also the theories proposed by 
Schlesinger in the Neurol. Centralblatt, 1892, 3). 

The least probable theory seems to be the one which as- 
sumes the disease to be of a peripheral nature. This can hardly 
be brought into accord with the fact that the affection has been 



TETANY. 



495 



known to follow psychical influences, for, just as we have seen 
to be the case in chorea, and as we shall soon learn for epi- 
lepsy, this disease also can be brought about by imitation, and 
indeed there have been instances recorded where in this man- 
ner even small epidemics of tetany appeared in schools (Mag- 
nan, Gaz. de Paris, 1876, 50, and Gaz. des hop., 1876, 141). 
The disease has further been observed in women who are suck- 
ling infants, in young mothers and wet nurses ; and so fre- 
quently has this been the case that Trousseau felt himself jus- 
tified in terming tetany " la contracture des nourrices." It has 
also been seen associated with various affections of the stomach 
especially dilatation. (Loeb, Deutsches Arch. f. klin. Med., 1889, 
xlvi, Heft 1, assumes that in such cases there occurs an absorp- 
tion of poisonous products which act upon the nervous system.) 
Quite inexplicable are those cases occurring after extirpation 
of goitres (N. Weiss, Falkson, v. Eiselsberg, and others) and 
after infectious diseases, especially scarlet fever and typhoid. 
All this speaks, however, in favor of the central nature of the 
disease, as does also the fact that the occupation may have 
some causative influence, inasmuch as people who have to use 
their arms, hands, and fingers a great deal — telegraph operators, 
seamstresses (Mader, Hirt) — are relatively frequently subject 
to it. According to our opinion, the cortical nature of tetany 
is as probable as the cortical nature of writer's cramp. In this 
connection it is to be noted that von Frankl-Hochwart has 
repeatedly observed psychoses developing in the course of 
tetany (Jahrbiicher f. Psych., 1890, ix, 1, 2). 

The great rarity with which the affection occurs makes it 
practically of little importance. If we add to this that the 
cases, which we see, run without exception a favorable course, 
one can understand why but little is to be said of the treatment. 
If any interference be necessary or desirable we may avail our- 
selves of the galvanic current, placing the anode over the af- 
fected parts and the cathode in some indifferent place. This 
may be repeated two or three times a week, each time a mod- 
erate current being allowed to pass for from three to five min- 
utes. During the attack this procedure is sometimes quite 
beneficial, whereas upon the course of the disease it has as little 
influence as the well-known nervines. We have used tepid 
baths with success, inasmuch as the patients felt very comfort- 
able in them and claimed to be able to notice a diminution in 
the frequency of the attacks. It is our opinion, however, that 



496 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

even the baths can be dispensed with, and that it is best not to 
subject the patient to any therapeutic measures at all. 

LITERATURE. 

Schultze, Fr. Ueber Tetanie unci die mechanische Erregbarkeit der peripheren 

Nervenstamme. Deutsche med. Wochenschr., 1882, 20, 21. 
Mader. Ueber die Beziehung der Beschaftigungskrampfe zur Tetanie. Wiener 

med. Blatter, 1883, 16. 
Lederer. Jahrb. f. Kinderheilk., 1883, xxii, 4. 

Baginsky. Tetanie bei Sauglingen. Archiv f. Kinderheilk., 1886, viii, 5. 
Meynert. Archiv f. Gyn., 1887, xxx, 3. 
Schotten. Berliner klin. Wochenschr., 1888, xxv, 14. 
Hoffmann (Heidelberg). Zur Lehre von der Tetanie. Deutsches Archiv f. 

klin. Med., 1888, xliii, 1. 
Frankl-Hochwart, v. Ueber mechanische und elektrische Erregbarkeit der 

Nerven und Muskeln bei Tetanie. Ibid., 1888, xliii, 1. 
Escherich. Idiopathische Tetanie im Kindesalter. Wiener med. Wochenschr., 

1890, 40. 
v. Jaksch. Klinische Beitrage zur Kenntniss der Tetanie. Zeitschr. f. klin. 

Med., 1890, xvii, 3, 4. 
v. Frankl-Hochwart. Die Tetanie. (From Nothnagel's Clinic.) Berlin, Hirsch- 

wald, 1 891. 
Eulenburg. Artikel " Tetanie " in Eulenburg's Real-Encyclopadie, 2. Aufl. 
Heim. Ueber Tetanie bei Gastrektasie u. s. w. Bonn, 1893, Inaug.-Dissert. 
Nicolajevic. Ueber die Beziehungen der Tetanie zur Hysteric Wiener klin. 

Wochenschr., 1893, vi, 29. 

Thomsen's Disease. — Under the name of Thomsen's disease 
an affection has been described which is characterized by " tonic 
spasms in the muscles during voluntary movements." When any 
muscle is moved voluntarily, as it contracts, a tonic, painless spasm 
comes on which either greatly impedes the intended movement or 
completely frustrates it. If the patient wishes to perform certain 
motions a sensation of fatigue is felt in the part and a resistance, 
which he has first to overcome before the intended movement can be 
executed. Objects which he is holding in his hands he can not let 
go at once and put down. If he opens his mouth, he can not close 
it without the aid of his hand (Fig. 148) ; he can not rise from his 
chair without assisting himself with his arms (Fig. 149). Running, 
dancing, gymnastics, the manipulations of the military drill, are 
absolutely impossible, and any such attempts distress him very 
much and bring him into the most annoying situations. If the 
musculature of the tongue is implicated a motor speech disturbance 
is added. Sensory disorders are not found, and in general the 
patients are perfectly well if they do not attempt to move. Ob- 
jectively may be noted, besides the increased excitability to the 
galvanic current, an unusually strong development of the muscula- 



THOM SEN'S DISEASE. 



497 



ture and an increased power which seem almost to belie the com- 
plaints of the patients that they are embarrassed in their movements. 
Heredity stands for a great deal in the disease, which was evi- 
dent from the first from the description which Thomsen himself gave 




Fig 148.— Thomsen's Disease. (After Charles K. Mills, of Philadelphia, International 

Clinics, April, 1891.) 



in 1876. He reported that in his own family in five generations more 
than twenty persons had suffered from it. Often it is congenital, 
hence Striimpell has proposed the name myotonia congenita. 

The nature of the malady is still a matter of conjecture. The 
fact that on galvanic stimulation of the muscles the contractures are 
slow and very prolonged, lasting even as much as thirty seconds — 
myotonic reaction of Erb — the observation of Erb that on micro- 
scopical examination the muscular fibres are seen to be broader, the 
nuclei multiplied, and the interstitial connective tissue increased, are 
not points sufficient to warrant a definite decision about the seat of 
the disease. Still, the possibility that we are actually dealing with 
32 



493 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



an affection of the muscles is by no means excluded. In favor of 
this latter view is the case reported by Dejerine and Sottas, in which 
changes were to be demonstrated only in the muscles (cf. Deutsche 
Med.-Ztg., 1893, 66, p. 741). 

The disease, which interferes greatly with the occupation, is wont 
to last throughout the entire life. The patients learn to accommodate 




Fig. 149.— Thomsen's Disease. (After Charles K. Mills.) 

themselves to a certain extent to the inconvenience, and by allowing 
for it are able in a measure to hide their awkwardness. In countries 
where military service is compulsory any one suffering from myotonia 
is exempt. No treatment has as yet been promulgated for this rarest 
of affections. 

LITERATURE. 

Thomsen (in Kappeln). Tonische Krampfe in willkiirlich bewegten Muskeln. 

Arch. f. Psych, u. Nervenkh., 1876, vi, 3. 
Weichmann. Ueber Myotonia intermittens congenita. Inaug.-Dissert, Vratisl., 

1883. 



THOM SEN'S DISEASE. 



499 



Mobius. Schmidt's Jahrbiicher, 1883, Bd. cxcviii, p. 236. 

Rieder. Deutsche militararztl. Zeitg., 1884, xiii. 

Pitres et Dallidet. Arch, de neurol., 1885, x. 

Eulenburg und Melchert. Berliner klin. Wochenschr., 1885, xxii, 38. 

Erb. Die Thomsen'sche Krankheit. Leipzig, Vogel, 1886. 

Fischer. Neurol. Centralbl., 1886, v, 4. 

Buzzard. Lancet, May 13, 1887, i, 20. 

Jacoby. Journ. of Nerv. and Ment. Dis., 1887, xiv, 3. 

Blumenau. Ueber die elektrische Reaction der Muskeln bei der Thomsen'schen 
Krankheit. Centralbl. f. Nervenheilk., 1888, xi, 22. 

Dana. Thomsen's Disease. Journ. of Nerv. and Ment. Diseases, April 4, 1888, 
N. S., xiii. 

Martius und Hansemann. Virchow's Archiv, 1889, cxvii, 3. 

Fleming. The Alienist and Neurologist, 1890, xi, p. 51. (Typical Non- 
congenital Myotonia.) 

Hughes. Ibid., p. 62. 

Dreschfeld. Thomsen's Disease. Brit. Med. Journ., February 22, 1890^.429. 

Hale White. Extract from the Guy's Hosp. Reports, 1890, xlvi, p. 329. 

Fries. Neurol. Centralbl., 1892, p. 40, 2. 

Delprat. Thomsen'sche Krankheit in einer paramyotonischen Famiiie. 
Deutsche med. Wochenschr., 1892, 83. 



CHAPTER III. 

PARALYSIS AGITANS SHAKING PALSY— PARKINSON'S DISEASE 

CHOREA PROCURSIVA. 

Among the diseases of which we are treating in this part 
paralysis agitans is the gravest, but happily also the rarest, for, 
according to statistics of my own cases, only about 0.43 per 
cent, or one in two hundred and twenty-nine, of all affections 
of the nervous system were instances of paralysis agitans. 
Within the some sixty years which have passed since Parkin- 
son's description appeared, certain symptoms of the disease 
have, it is true, been studied more carefully, but our knowl- 
edge of the aetiology, the anatomical seat, the treatment, etc., 
has not improved to any extent, and in fact our progress has 
been unsatisfactory. 

Symptoms. — The first thing observed by the patient is a 
feeling of weakness in the extremities, followed soon after by a 
slight tremor, which at first only occurs temporarily. It is 
more marked in the upper extremities, especially in the right 
arm, yet it is also noticeable in the legs, and exceptionally in 
the head. The old idea that the head is always exempt from 
the tremor of paralysis agitans, and that this exemption is, 
ccBteris paribus, characteristic of the affection, is untenable. In 
rare instances the tremor is confined to one half of the body, 
whereas the other remains quiet. 

The tremor consists of uniform oscillating movements, the 
oscillations being rather few in number, not more than from 
four and three quarters to five and a half per second (Cramer), 
whereas the tremor of Graves' disease, for example, presents 
from nine to nine and a half oscillations per second (Marie). 
The lengths of the oscillation waves have been studied by Marie, 
Cramer, and others, and the handwriting of the patients has gen- 
erally been utilized for such observations. The oscillations were 
recorded on paper by means of a Marey's drum or rubber ball, 
500 



PARALYSIS AGITANS. 



50I 



which the patient was made to hold loosely in his hand. Re- 
peatedly with perfect regularity of the wave lengths a varia- 
bility in their height could be demonstrated, the physiological 
cause for which is not entirely clear. It is not infrequently 
seen that the tremor increases on forced attempts at motion, 
and passes into a regular " shake," so that the patient, although 
not entirely helpless, becomes very awkward in feeding himself. 
It is a fact of considerable diagnostic importance that the 




'**?«*£* 



Fig. 150.— Specimen of Handwriting of Patient with Paralysis Agitans (personal 

observation). 



movements during rest in bed do not cease, but continue and 
hinder the patient from getting to sleep, and (in contradistinc- 
tion to what we find in chorea) do not disappear even during 
sound slumber. Indeed, the intensity of the tremor may remain 
undiminished in bed, and I know of instances in which the pa- 
tients procured for themselves iron bedsteads in order to avoid 
the annoying creaking of the wooden bed caused by the vio- 
lent shaking. In other cases the condition improved upon 
lying quietly in bed, and falling asleep was facilitated by the 
use of certain artifices. Thus Eichhorst relates of one of his 
patients that he always carried a little twig between his teeth 
so as to keep his jaws quiet, and one of my own cases only 



502 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

could obtain comfort and a certain amount of rest in his fingers 
and arms by rolling small objects — for instance, little wooden 
balls which he had made for the purpose — between his fingers 
and thumb. With the aid of these he also could go to sleep. 
If by accident he left these balls at home, he unconsciously 
picked up other objects which might be lying before him, such 
as matches, or he rolled bread pellets, and so on, and only felt 
comfortable when his fingers were occupied with something 
of this sort. The change in the handwriting caused by this 
tremor is illustrated in Figs. 150 and 151. 

In connection with, and probably as a consequence of, the 
trembling movements, gradually a condition develops in which 

// fa ft. 9ut*& 2M 

Fig. 151. — Specimen of Handwriting of Patient with Paralysis Agitans (personal 

observation). 

the patient gets easily tired, the muscular strength diminishes, 
and the muscles assume a certain rigidity which influences the 
position of the body and the extremities when at rest as well 
as on voluntary motion. The position of the body is charac- 
teristic. Not only the head, which affords a good deal of re- 
sistance to passive movements, but also the whole trunk is 
somewhat bent over, and it appears as if the patient was at 
every moment ready to fall forward. The arms, which are 
bent at the elbows, are in close apposition to the trunk, the 
thumb rests against the fingers, so that the hand assumes some 
such position as it would in writing, the fingers themselves 
being flexed in the metacarpal joints (Fig. 152). The knees 
are so close together that the trousers are rubbed agair.st each 
other by the trembling movements, and walking is not a little 
interfered with. The legs are usually slightly flexed at the 
knee joints, while nothing remarkable can be noticed about 
the joints of the toes. The patient impresses one as being in 
a constant state of uncertainty and perplexity, an impression 
which is only diminished to a certain degree by the very char- 



PARALYSIS AGITANS. 



503 



acteristic facies. The rigidity being also marked in the mus- 
cles supplied by the seventh nerve, the face has an expression 
of majestic calm, nay, even of sublimity. The patient seems 
inaccessible to psychical emotions. His smile is hardly per- 
ceptible, since the lower portions of the face more especially 
are almost immobile. Only the wrinkling of the forehead is 
somewhat more marked. Sometimes the patients have a 
peculiar piping voice, such as an actor assumes when playing 
the part of an old man on the stage. 

We have already alluded to the fact that voluntary move- 
ments are somewhat impeded. This is due not only to the 
tremor, but also to the already-mentioned general weakness. 




Fig. 152.— Position of Hands and Fingers in Paralysis Agitans (as if holding a 
pen). (After Eichhorst.) 

He is, therefore, helpless, and needs some one to assist him if 
he wishes to sit up in bed or even to change his position. If 
he is in a sitting posture rising is difficult, sometimes impos- 
sible. The act of walking is not normally performed, for be- 
sides the bent position, which in walking becomes even more 
exaggerated than in standing, the patient once started has an 
irresistible tendency, owing to the displacement of his centre of 
gravity forward, to hurry ahead ; his steps, at first short and 
tripping, become quicker and longer, and so great may be the 
force with which he involuntarily rushes forward that if there 
is no one there to stop him he falls on his face with great vio- 



504 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



lence. The same phenomenon, which is called " propulsion," 
may sometimes be artificially produced by pulling the patient 
forward by the coat while he is walking quietly. He then 
goes faster and faster, and finally breaks into a run alarming to 
the bystanders. Much more rarely do we find a similar con- 
dition in the backward motion (" retropulsion "), so that the 




Fig- 153.— Position of the Body in Paralysis Agitans (personal observation). 



patient if pulled from behind walks backward faster and faster, 
to fall over in a short time. Charcot looks upon these phe- 
nomena as forced movements, a view which has, however, 
never been substantiated. They may possibly be explained on 
purely physical grounds as being due to the displacement of 
the centre of gravity of the body (Strumpell). 

Trophic changes, with the exception perhaps of the tran- 
sient appearance of purpuric spots symmetrically on the arms 
and legs (" senile purpura "), are not met with. Changes in 
the electrical excitability of the muscles do not occur, or are, 
at any rate, not the rule. Sensation and reflexes remain entirely 



PARALYSIS AGITANS. 505 

normal, and bladder as well as rectal symptoms are not pres- 
ent. An increase in the body temperature can never be dem- 
onstrated objectively, although patients complain at times of 
subjective feelings of increased heat and a disagreeable tend- 
ency to sweat a good deal, which is especially pronounced 
when lying in bed, so that they often sleep uncovered or with 
but little over them. If any cerebral or spinal symptoms make 
their appearance these have to be regarded as complications. 
They do not belong to the clinical picture of paralysis agitans 
as we know it now. 

Cases in which muscular weakness and rigidity, with all 
their inconvenient consequences, were present, in which, also, 
the so-called propulsion was marked, but the tremor was ab- 
sent, have been reported (Amidon, New York Medical Record, 
1883, xxiv, 21), but such are rare. 

The nature of the disease is not yet understood. We do 
not even know whether to refer it to the brain or to the mus- 
cles. Much less, of course, do we know where the exact seat 
should be sought for in the nervous system. Before the labors 
of Charcot and Ordenstein, paralysis agitans was often con- 
founded with multiple sclerosis, and various anatomical lesions 
were then described as underlying the paralysis agitans. Later 
the error was cleared up, and even to the present day we are 
not acquainted with any anatomical basis for the disease. 

^Etiology. — In this respect also our knowledge is very in- 
complete. Of course here, as in all other nervous diseases, he- 
redity and the importance of a neuropathic family history must 
be spoken of, yet the rarity with which the affection occurs shows 
that this factor alone is seldom sufficient to cause the disease. 
Hence other exciting causes must come into play, but it is a 
fact difficult to understand why the same factors which so often 
give rise to chorea so rarely produce a shaking palsy. The 
causes for all these diseases are always the same, or at least 
similar, and it is here also in the first place that psychical emo- 
tions of fright and anxiety are of moment. The French phy- 
sicians have at no time seen develop so many cases of paraly- 
sis agitans as during the time of the siege in 1871, and for years 
after the relative frequency of the trouble in the Paris hospi- 
tals, particularly in the Salpetriere, acted as a reminder of the 
terrible hours which the besieged must have gone through. 
In private practice we also have occasion to find that psychical 
causes bring about the disease ; more frequently, however, at 



506 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

least in my own experience, no cause at all can be found. The 
influence of exposure to cold and of overexertion of course has 
here also been thought to be of serological significance with- 
out there being any grounds for such an assumption; on the 
other hand, there is no question but that certain infectious dis- 
eases — e. g., intermittent fever, pertussis, typhoid fever — may be 
followed by a paralysis agitans, a connection, however, which, 
although certain in its existence, is still obscure in its nature. 
Nothing definite is known about the influence of age and sex. 

Diagnosis. — After what has been said little needs to be 
added with regard to the diagnosis, which is almost always 
easy. It is certainly not hard to avoid mistaking paralysis 
agitans for multiple sclerosis or chorea, and chronic alcoholism 
is easily excluded if we take into account the characteristics of 
the tremor, its continuance during sleep, and the whole course 
of the disease. It may be sometimes difficult to differentiate a 
shaking palsy from the ordinary tremor senilis if the latter 
occurs as early as the forties, at a time of life during which 
paralysis agitans is not rare, and it is the more necessary to be 
careful, since the number of the oscillations in both affections 
is about the same — that is, ranges between four and six per 
second. The muscular weakness, the peculiar rigidity which 
accompanies the movements, the characteristic facial expres- 
sion, the posture, the " propulsion," etc., will in most cases be 
sufficient to clear up the diagnosis. Oppenheim has observed 
that the so-called traumatic neurosis may present the picture of 
paralysis agitans (Pseudo-Paralysis Agitans ; Charite-Annalen, 
1889, xiv, p. 418). 

Treatment. — The treatment is entirely fruitless. We have 
not as yet seen any results from any of the therapeutic meas- 
ures employed. Neither with baths nor with massage (Ber- 
bez, cf. lit.) nor with galvanism has anything been achieved, 
and all internal medicines are of no avail. It is impossible to 
give particular indications for the treatment, and it must there- 
fore remain for the physician in every case to treat alternately 
with baths, massage, and electricity, according as he sees fit. 
As long as he does not do the patient any harm, it does not 
matter much which mode of treatment he decides to use. 
Lately Erb has recommended the muriate of hyoscine injected 
subcutaneously or taken internally. This is said to exert a very 
good influence upon the tremor, but whether this effect is last- 
ing, and whether the bad after-effects which occasionally appear 



MIGRAINE. 



507 



after a prolonged use of the drug" are not a grave objection to 
its administration, is not as yet decided. My own experiences 
with it were not favorable. Charcot's " vibration treatment," 
by which a quieting or even benumbing effect is aimed at, was 
further studied by Gilles de la Tourette (Progres med., 1892, 
35). This author has constructed a special apparatus in the 
shape of a helmet. Five thousand to six thousand vibrations a 
minute are said to produce a hypnotizing effect and to diminish 
the tremor. I am inclined to think that the result is chiefly 
due to suggestion. 

LITERATURE. 

Heimann. Ueber Paralysis agitans. Berlin, Hirschvvald, 1888. 

Lacoste. Contribution a l'etude de la maladie de Parkinson. These de Paris, 

1887. (De quelques formes anormales.) 
Huber. Myographische Studien bei Paral. agit. Virchow's Arch., 1887, 108, 1, 

p. 45- 
Teissier. Pathogenie de la paralysie agitans. Lyon med., 1888, lviii, 28. 
Weber. Paralysis Agitans, with Cases. Joum. of Nerv. and Ment. Diseases, 

July 7, 1888, N. S., xiii. 
Dutil. Sur un cas de Paralysie agitans a forme hemiplegique, avec attitude 

anormale de la tete et du tronc. Gaz. med. de Paris, 1889, 38, p. 449. 
Martha. Etude clinique sur la paralysie agitans. Paris, Steinheil, 1888. 
Peterson. A Clinical Study of Forty-seven Cases of Paralysis Agitans. New 

York Med. Journ., October 11, 1890. 
Hadden. Paralysis Agitans in a Young Man. Brain, 1890. 
Sass. Petersburger med. Wochenschr., n. F., 1891, viii, 19, 20. 
Koller. Virchow's Archiv, 1891, cxxv, p. 287. 
Leva. Deutsche Zeitschr. f. Nervenhk., 1891, ii, 1. (Condition of the Urine in 

Paralysis Agitans.) 
Ketscher. Zeitschr. f. Heilkunde, 1893, xiii, 6, p. 445. 
Dana. New York Med. Journ., 1893, 57, No. 23. 

B. AFFECTIONS IN WHICH THE SENSORY NERVES ARE CHIEFLY 

IMPLICATED. 

The only affection which can at present be assigned to this 
group is one which deserves a good deal of attention, on ac- 
count not only of its frequency, but also of the obscurity which 
still exists with regard to its pathogenesis. It is a malady 
which never seriously endangers the patient's life, but never- 
theless produces grave, almost unbearable, suffering. 

Migraine {Hemicranid). 

The disease manifests itself in attacks, while in the interven- 
ing periods the patients are usually perfectly well and in no 
way give evidence of the severity of the affliction of which 



508 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

they are the subjects. The paroxysms are usually preceded 
for several hours by prodromal symptoms, general lassitude, 
chilly feelings, a tendency to yawn, buzzing in the ears, and the 
like. If the regular attack is going to begin in the morning, 
the patient wakes up repeatedly during the night, and is thus 
able to predict with certainty that the headache is coming on. 
The pain is sometimes confined to one side of the head, and, 
according to statistics, the left seems to be the one more com- 
monly implicated ; but the seat often changes during the attack, 
so that the patient complains now of the left, now of the right 
side of the head. Sometimes a distinct pallor is noticeable on 
one side during the attack, associated with dilatation of the 
pupil and increase in the salivary secretion, while in other in- 
stances one half of the face is flushed and hot, the arteries pul- 
sating strongly, and the pupil contracted. In the first case we 
designate the hemicrania as spastic (sympathico-tonica, con- 
nected with stimulation of the sympathetic) ; in the latter as 
paralytic (connected with paralysis of the sympathetic). The 
former has been described by Du Bois-Reymond, the latter by 
Mollendorf, in both cases after observations made upon them- 
selves. But these conditions are not constant either, and if one 
has seen many attacks of migraine he knows full well that the 
patients often change color — they are now pale, now flushed, 
now complain of a feeling of heat in the head, now of cold. 

If the pain is very violent the patient shows general con- 
stitutional symptoms. In a bad attack he lies for hours com- 
pletely apathetic, meeting every question and every source of 
disturbance with unmistakable signs of disgust. He refuses 
nourishment entirely, owing to a feeling of utter discomfort 
and an almost uncontrollable desire to vomit. Only after copi- 
ous vomiting of bile-like mucoid masses does his condition 
gradually improve, the amelioration beginning with a violent 
desire for food and a polyuria following the attack, which is 
finally ended by a refreshing sleep. When vomiting does not 
occur the patient suffers for a longer period. Sometimes the 
eyes participate, and photophobia, flitting scotomata, even 
hemianopia, have been observed during the attack. These 
are instances of the type which Fere, Galezowski, Dardignac, 
and others have described as migraine ophthalmique. In place 
of the flitting scotomata, visual hallucinations are observed in 
exceptional cases (Weir Mitchell, Araer. Jour. Med. Sci., 1887, 
October, p 415). 



MIGRAINE. 



509 



It is not uncommon for the attacks not to reach their full de- 
velopment ; in which cases only certain symptoms — flitting sco- 
tomata, vomiting-, vaso-motor disturbances, or the like — may 
appear. Such isolated symptoms may be called " hemicranic 
equivalents " (Mobius). 

The duration of the attack varies from a few hours to a 
whole day ; it rarely lasts longer, and if it does, this fact should 
always make us doubtful as to the diagnosis. In the intervals 
the patients as a rule feel well ; still, if the attacks are very se- 
vere and frequent, occurring, for instance, as often as once or 
twice a week, the after-effects may be so lasting that the suffer- 
ers never enjoy perfect health. Indeed, the attacks may occur 
with such frequency that we have what Fere calls etat de vial 
migraineux and Mobius status hemic? -aniens, a condition in which 
transitory psychoses may develop (Zacher, Berliner klin. Wo- 
chenschrift, June 11, 1892). Fortunately, such a rapid succession 
of the seizures is uncommon. Once a month or six or eight 
times a year is the rule, not counting slight, abortive attacks. 

The course of migraine is always extremely tedious, some- 
times lasting through a whole lifetime. In women the climac- 
teric period occasionally, but by no means always, exerts a 
beneficial influence. At the time of menstruation the attacks 
seem to be especially apt to occur ; and even if no definite at- 
tack makes its appearance, women who are subject to migraine 
complain of more or less severe headaches at such periods. 
Not infrequently the disease has an unfavorable influence on 
the disposition and appearance of the patients ; they become 
peevish and ill-tempered, and even in the intervals between the 
attacks are by no means amiable or sociable. They are wont 
to restrict themselves considerably in their social intercourse — 
for one reason, because they are rarely able to make engage- 
ments for definite times on account of the possibility of the 
occurrence of one of their attacks. The trophic disturbances 
which are sometimes superadded, as, for instance, the prema- 
ture gray hairs, make such patients look older than they really 
are ; on the other hand, there are individuals who, notwith- 
standing the severity of the attacks, retain for a long time their 
youthful freshness and vivacity. 

With regard to the pathological anatomy and the pathogen- 
esis we know scarcely anything ; it appears not unlikely that 
the brain cortex more especially and its sensory elements are 
primarily the seat of the affection, and it seems more and more 



[JIO DISEASES OF THE GENERAL NERVOUS SYSTEM. 

probable that, besides the influence which must be attributed to 
heredity, here too, as has been claimed for certain cases of epi- 
lepsy, auto-intoxication is to be regarded as a not improbable 
factor. But it must be admitted that this is only a supposition, 
and that we are without any certain knowledge on this point. 

Recovery, if it ever occurs, is certainly very rare, and can 
probably never be regarded as the result of treatment. If 
aphasia or motor disturbances are persistently associated with 
hemicrania, the latter is to be regarded merely as a symptom of 
an underlying organic disease, and nothing definite can be said 
with regard to the prognosis. In this connection must be men- 
tioned the case of Oppenheim, in which a thrombus of the in- 
ternal carotid artery was found to be the cause of the headache 
and of the other symptoms (Charite-Annalen, xv, Jahrg.). The 
prognosis is relatively favorable if in the intervals between the 
attacks the patient enjoys sound and healthful sleep. Unfortu- 
nately, in the majority of cases they are deprived of this, and 
in order to procure it are forced to resort to artificial means, of 
which the bromides are the most popular. It is not always easy 
to understand the cause of the sleeplessness (agrypnid) in migraine, 
and for that matter in all nervous diseases ; it is especially diffi- 
cult to do so when this is the only symptom and absolutely 
nothing else can be detected, when individuals otherwise healthy 
are wholly or almost wholly deprived of sleep for weeks ; and 
yet it is just the discovery of this primary cause that is of the 
greatest importance, as it will guide our action in the treat- 
ment ; and only when this is found can we reasonably hope for 
improvement from our efforts. Sometimes we have to deal 
with a gastric catarrh which until this time has been over- 
looked, a hyperaemia of the liver, and the like, and after the 
successful treatment of these by Carlsbad water, etc., sleep, 
which in spite of all bromides and morphine has in vain been 
sought, returns of its own accord. Sometimes a marked grade 
of anaemia may lie at the bottom, easily recognizable by the 
pallor of the skin, the small pulse, and the cold extremities. 
In such cases cod-liver oil, iron, and quinine are more service- 
able than the usual hypnotics, which are rarely well borne. 
In all nervous patients suffering from insomnia it is advisable 
to examine the thoracic and abdominal as well as the sexual 
organs, and only to treat the sleeplessness symptomatically 
when repeated examinations have given negative results. This 
symptomatic treatment consists above all in the careful use of 



MIGRAINE, 



511 



massage, which should be supervised by the physician, a prac- 
tice from which we have obtained very gratifying results. 
Next comes the systematic galvanization of the brain, for the 
technical details of which the reader is referred to my book on 
electro-diagnosis, pp. 186 et seq. As a last resort we have the 
administration of quieting, calming, and sleep-producing drugs, 
among which, notwithstanding all the new hypnotics, morphine 
still holds the first place. Besides this, chloral, paraldehyde, 
urethan, hypnone, coniine, lupuline, sulfonal, and amylene hy- 
drate (tertiary amyl alcohol), which has recently been recom- 
mended by von Mering, may be tried. The last is best given 
in doses of three and a half to four grammes (tiiI-Ix) in one 
dose once in twenty-four hours, and seems often to have a favor- 
able action. On account of the bad taste of this drug the addi- 
tion of correctives — for instance, the oil of peppermint, which 
somewhat masks the taste — is to be recommended. (Amylene 
hydrate, 7.0 (^cv); aq. menth. pip., 40.0 ( ~ x) ; ol. menth. pip., 
i.o(TTixv); syrup, simpl., 30.0 (5j)- Sig. : Half to be taken at 
night.) The sleep after it is deep and quiet, and unpleasant 
after-effects are rare. Nevertheless, it is well to be careful in 
its administration, as symptoms of intoxication may appear, as 
Dietz has reported (Deutsche Medicinal-Zeitung, 1888, 18). Tri- 
onal has been recommended by Schultze (Therap. Monatsch., 
1891, October); its effect has also been studied by Brie (Neu- 
rol. Centralbl., 1892, 24), who has found it very useful in doses 
of from 1 to 2 grammes (15 to 30 grains), without noting any bad 
after-effects. The reports with regard to methylal and chlo- 
ralamid are still conflicting (cf. lit.). 

The medicinal as well as the general treatment of migraine 
is, on the whole, the same as that of habitual headache, which 
has already been discussed on page 65. It may be added that 
the so-called migranin, a combination of antipyrin, citric acid, 
and caffein, in certain proportions, prepared by Overlach, is 
deserving of further trial (^Deutsche med. Wochenschr., 1893, 
xix, 47). 

LITERATURE. 
1. Migraine. 
Kroll. Berliner klin. Wochenschr., 1888, 30. 
Galezowski. Progres med., 1888, 29. 
Dardignac. Revue de med., October 10, 1888, viii. 
Neftel. Deutsche Med.-Ztg., 1890, 14, p. 157. 
Benedikt. Wiener med. Presse, 1892, 8. 
Haig. Brain, November, 1893, p. 230. 



512 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



2. Insomnia arid Hypnotics. 
Lemoine. Gaz. med. de Paris, 1887, 18. (Methylal.) 
Personali. L'Union med., 1887, 9. (Methylal.) 
Illingworth. Insomnia. Med. Press and Circ, August 29, 1888. 
Reichmann. Chloralamid, ein neues Schlafmittel. Deutsche med. Wochenschr., 

1889, 31. 
Jastrowitz. Schlaflosigkeit. Ibid., 1889, 31. 

Mauthner. Schlafkrankheiten. Allg. Zeitschr. f. Psychiatr., 1891, xlvii, 6. 
Brie. Trional als Schlafmittel. Neurol. Centralbl., 1892, xi, 24. 
Filehne. Ueber das Hypnal-Hochst. Berliner klin. Wochenschr., 1893, 

xxx, 5. 
Koppers. Wirkung des Trionals. Inaug.-Dissert., Wiirzburg, 1893. 



C. AFFECTIONS IN WHICH THE TROPHIC NERVES ARE CHIEFLY 

IMPLICATED. 

Our acquaintance with the few affections to be described 
under this head is of very recent date. Since their pathogene- 
sis and their seat are as yet obscure, and since we have to con- 
fine ourselves to the 
description of the 
most striking symp- 
HP' toms, it is impossi- 
ble to say whether 
the place here as- 
signed to them is 
correct or not. 



1. Acromegaly {P. Marie). 

Under the name of acro- 
megalia (aKpov, extremity) 
Marie described, in 1886, a 
peculiar non-congenital hy- 
pertrophy of the hands, feet, 
and head, to which affection 
attention had previously 
been drawn by Fritsche and 
Klebs (cf. lit.). The extrem- 
ities appear increased in 
length as well as in breadth. 
The bones of the face, espe- 
cially those of the cheeks 
and the lower jaw, present 
considerable enlargement 
(cf. Fig. 154), and the meas- 




Fig. 154. — A, the lower jaw of a patient under 
the care of Professor Marie, in Paris. B, a 
lower jaw which normally would correspond 
to the size of the patient. 



ACROMEGALY. 



513 



urements of the skull are above normal. In the same way the 
lips, ears, nose, and tongue are found enlarged, whereas all the 
muscles are feeble. The skin appears yellowish and pale, but 
is otherwise normal. The thyroid gland was almost always 
very atrophic in the cases observed up to the present time. 




Fig. 155.— Case of Acromegaly. (After P. Marie.) 

In spite of their gigantic appearance the patients are feeble 
and without strength. The sexual functions are lost early and 
completely (Freund, cf. lit.). 

The onset of the disease dates back to early childhood, and 
it has to be regarded as an abnormity in development (Freund) 
" which, probably beginning as early as the cutting of the 
second teeth, certainly sets in energetically at the period of 
puberty, and consists in a rapidly developing enlargement of 
the facial part of the skull, which by far exceeds the physio- 
logical limits of growth. This increase is especially marked 
in the lower jaw and also in the extremities, with their girdle 
attachments, while the rest of the skull and the trunk are only 
secondarily altered." The observation of Gerhardt, whose 
patient was perfectly well up to his sixtieth year, does not agree 
with this view (Berliner klin. Wochenschr., 1890, 52). 

A relatively large number of cases have come to autopsy 

33 



5 i 4 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

since Marie's publication, and from the number of instances 
reported the disease would seem to be by no means rare. The 
results of these autopsies have not been very satisfactory, for 
besides a more or less pronounced increase in the volume of 
the hypophysis (Boltz, Gauthier, Holsti), nothing worthy of 




Acromegaly. (After Buchwald ) 



note has been found ; and since we know nothing of the func- 
tion of the hypophysis, this finding has thus far proved of little 
value for the understanding of the pathogenesis of the disease. 
Nor is our information any more satisfactory so far as cause 
and treatment are concerned. 

According to Goldscheider, who established the fact that 
the giant growth is not confined to the distribution of any 



ACROMEGALY. 



5^5 



special nerve, being- found, for instance, in the hand, in that 
of the musculo-spiral and the median, more rarely in that of 
the ulnar, the aetiological influence of the trophic fibres is still 
a matter of doubt. Pel (Berliner klin. Wochenschr., 1891, 3) 
observed a case in which psychical traumatism during men- 
struation was followed by acromegaly. 

LITERATURE. 

Fritsche und Klebs. Beitrag zur Pathologie des Riesenwuchses. Leipzig, 
1884. 

Marie. Surdeux cas d' Akromegalie. Revue de med., 1886, iv, 4, p. 297. 

Frantzel, O. Deutsche med. Wochenschr., 1888, 32. 

The same. Deutsche Med.-Ztg., 1888, 48, p. 581. 

Erb. Deutsches Arch. f. klin. Med., Februar 4, 1888, xvii. 

Broca. Un squelette d'Akromegalie. Arch. gen. de med., December, 1888. 

Adler. Boston Med. and Surg. Journ., November 21, 1888, cxix. 

Freund, V. A. Ueber Akromegalie. Volkmann'sche Sammlung klin. Vortrage, 
1889, 329, 330 (11. Ser., Heft 29, 30). 

Virchow. Ein Fall und ein Skelett von Akromegalie. Berliner klin. Wochen- 
schr., 1889, 5. 

Goldscheider. Arch. f. Anat. u. Physiol., Physiol. Abtheilung, 1889, 1, 2. 

Saundby. Acromegaly. Brit. Med. Journ., January 5, 1889. 

Marie, P. L' Akromegalie. Etude clinique. Paris, 1889. 

Rolschewnikoff. Ein Fall von Syringomyelic und eigenthumlicher Degenera- 
tion der peripheren Nerven, verbunden mit trophischen Storungen. 
(Akromegaly.) Virchow's Archiv, 1890, cxix, Heft 1, p. 10. 

Recklinghausen, v. Ueber die Akromegalie. Nachschrift zur vorstehenden Ab- 
handlung. Ibid., 1890, p. 36. 

Tanzi. Rivista clinica, 1891, Heft 5. 

Litthauer. Deutsche med. Wochenschr., 1891, 47. 

Duchesneau. Contribution a l'etude anatomique et clinique de l'Al<romegalie. 
Paris, Bailliere, 1892. 

Murray, G. Acromegaly. Brit. Med. Journ., February 27, 1892. 

Arnold. Akromegalie, Pachyakrie oder Ostitis ? Ziegler's Beitr. z. pathol. 
Anat., 1892, x, Heft 1. 

Boltz. Ein Fall von Akromegalie mit bitemporaler Hemianopsie. Deutsche 
med. Wochenschr., 1892, 27. 

Gauthier. Progres med., 1892, 1. 

Holsti. Ein Fall von Akromegalie. Zeitschr. f. klin. Med., xx, 4-6. 

Gause. Deutsche med. Wochenschr., 1892, 40. 

Fratnich. Allg. Wiener med. Ztg., 1892, 37. 

Souques et Gasne. Nouv. Iconograph. de la Salpetriere, 1892, 5. 

Gessler. Med. Correspondenbl. d. Wiirtemb. arztl. Landesvereines, lxiii, 1893, 
16. 

Arnold. Weitere Beitrage zur Akromegaliefrage. Virchow's Archiv, 1894, 
cxxxv, Heft 1 (with numerous references). 



5 16 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

2. Osteoarthropathy. 

Another affection depending on trophic alterations, which 
in milder cases also manifests itself by changes in the hands 
and feet, was described in 1890 by P. Marie under the name 
osteoartJiropathie hypertrophiante pneumique. The condition is 
characterized by a colossal increase in the finger nails, the 
terminal phalanges of the fingers and toes becoming thick- 
ened, and the nails assuming a shape which, when seen from 
the side, remind one of a parrot's beak (cf. Fig. 157). The 
resemblance which the fingers bear to drumsticks justifies 
the term "drumstick fingers" (cf. Fig. 158). In more pro- 
nounced cases the ends of the bones of the forearms and of 




Fig. 157.— Osteoarthropathy. (After Rauzier; Revue de med., 1891, ii, 1.) 

the tibia and fibula also become thickened. The fundamental 
difference between osteoarthropathy and acromegaly lies in 
the fact that in the latter we have an enlargement of all the 
terminal portions of the extremities as well as of the face. The 
aetiology of the affection, according to Marie, is to be sought 



OSTEOAR THROPA THY. 5 j 7 

in the existence of pulmonary affections in which extensive de- 
composition of pus occurs, for which reason this writer has 




Fig. 158.— Osteoarthropathy. 
(After Spillmaxn and Haushalter; Revue de med., 1890, x, 5.) 

employed the term pneumique. For details in connection with 
this affection the reader is referred to the special articles men- 
tioned below. 

LITERATURE. 

Marie. Revue de med., 1890, x, 1 (the original article). 

Spillmann et Haushalter. Ibid., 1890, x, 5. 

Lefebre. These de Paris, 1891. Des deformations osteo-articulaires consecu- 

tives a des maladies de l'apparael pleuro-pulmonaire. 
Gerhardt. Berliner klin. Wochenschr., 1890, xviii, 52. 
Arnold. Beitr. z. path. Anat. u. allg. Path., 1891, x, 1. 
Rauzier. Revue de med., 1891, xi, 1. 
Mobius. Munch, med. Wochenschr., 1892, 23. 
Minor. Neurol. Centralbl, 1893, 16, p. 565. 



5 18 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

APPENDIX. — I. GRAVES' DISEASE — BASEDOW'S DISEASE (GLOTZAU- 

GENKRANKHEIT, CACHEXIE EXOPHTH ALMIQUE) EXOPHTHALMIC 

GOITRE — TACHYCARDIA STRUMOSA EXOPHTHALMICA. 

This condition, first described by Parry, later by Graves, 
and which in Germany is generally known as Basedow's dis- 
ease, is an affection of the general organism in which certain 
symptoms referable to the central nervous system are, as a 
rule, the most prominent features. According to our present 
conceptions, which are, however, not fully established, exoph- 
thalmic goitre can not be regarded as a disease of the nervous 
system in the stricter sense, since the anatomical seat of the 
affection is situated not in the nerve tissues but in the thyroid 
gland. The anatomical changes which have thus far been 
found in the nervous system (Sattler, Filehne, and others) are 
not constant, and, as it seems, not essential for the pathogenesis 
of the disease. 

Symptoms. — The three symptoms which are regarded as 
characteristic of Graves' disease are (i) an excited, accelerated 
action of the heart, with visible pulsation in the arteries of the 
neck ; (2) enlargement of the thyroid gland ; (3) exophthalmos. 
As a rule, the heart symptoms are the first to appear. The 
increase in the frequency of the pulse is variable. We may 
count from a hundred to a hundred and fifty beats a minute, 
and not infrequently the intensity of the heart beat is more 
forcible than normal, a circumstance which adds much to the 
discomfort of the patient. Auscultation does not always re- 
veal abnormalities. Occasionally a systolic souffle is audible, 
but this is often absent. Enlargement of the heart also has 
been observed. The extraordinarily strong pulsation in the 
carotids, which is very conspicuous and easily felt, is in re- 
markable contrast to the smallness of the pulse wave in the 
radial artery (Parry). 

The swelling of the thyroid is rarely very great. It is usu- 
ally symmetrical. In the gland itself pulsation can be easily 
seen, and on palpation a distinct thrill is communicated to the 
hand. I may say that I have repeatedly seen cases in which 
the volume of the gland changed from time to time, and that 
this change became perceptible in a comparatively short time, 
sometimes even in a few hours. 

An arterial souffle is heard over the gland, the cause of 
which is to be sought in a hypertrophy of the left ventricle 



GRAVES' DISEASE. 



519 



and a disproportionate enlargement of the thyroid artery 
(P. Guttmann, Deutsche med. Wochenschr., 1893, 11). 

The exophthalmos, which is probably always bilateral, also 
differs in degree in different cases. In the majority, however, 
it is so marked that the protruding eyeballs can not be com- 
pletely covered by the lids during sleep. This gives to the 
patient an appearance which to the layman is both peculiar 
and repulsive (Fig. 159), and is still more aggravated if the 




Fig. 159. — Graves' Disease ^personal observation). 

upper eyelid does not follow the downward motion of the 
ball, and thus allows a zone of the sclerotic, 1 to 2 mm. in 
width, to become visible above the cornea. This defective co- 
operation of the lid and ball (Graefe's symptom), which hap- 
pily does not occur very often, makes the patient frequently 
an object of horror to those about him. The almost com- 
plete absence of the involuntary winking of the lid (Stellwag's 
symptom) is quite conspicuous, especially since the voluntary 
movements can be made as well as before. We can then easily 
understand that our patients, particularly when they are ladies 
of the better classes, avoid as far as possible the contact with 
friends and acquaintances, as well as with strangers. 



520 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Ophthalmoscopically only one characteristic sign has been 
noted — namely, the spontaneous pulsation of the retinal vessels, 
discovered by O. Becker. This is not confined to the disk, 
but can be observed in the retina as well. With this exception 
there are no changes in the fundus, and eyesight, accommoda- 
tion, and pupils are entirely normal. Only on the cornea we 
occasionally find a decrease in sensibility, probably due to the 
want of moisture on the ball, the normal quantity of the lachry- 
mal fluid not being sufficient on account of the undue evap- 
oration which takes place, because the two lids are far apart, 
and winking only rarely occurs (Berger, Arch. d'Ophth., 1894, 
Fevrier). 

Insufficiency of convergence, a symptom first described by 
Mobius, is sometimes observed. If the patient be asked to 
look at a near point, one eye will soon be found to deviate out- 
ward. 

Narrowing of the field of vision has been described by 
Kast and VVilbrandt (Arch. f. Psych., 1890, xxii, 2). 

Among the subjective symptoms, in addition to the annoy- 
ing palpitation already mentioned, a tendency to free perspira- 
tion may be noted. Even slight exertion produces a feeling of 
heat, more especially in the head and neck, so that the patient 
preferably remains in cool, shady places, and sleeps with as 
little covering as possible, etc. An actual elevation of temper- 
ature is, however, not always objectively demonstrable. This 
tendency also accounts for the blushing evoked by the least 
bodily exertion or mental emotion. Both symptoms I have 
not infrequently seen to occur unilaterally. Trousseau has 
mentioned the fact that the most gentle stimulation of the skin 
of the face and neck produces a deep-red mark, designated by 
him as tache cerebrate, a phenomenon, however, which can ap- 
parently not always be evoked. All these symptoms are at- 
tributable to asthenia of the vaso-motor nerves, as is also the 
decrease in the resistance which the skin offers to the electri- 
cal current, first observed by Charcot (the Charcot- Vigouroux 
symptom), the saturation of the skin with fluid resulting 
from the dilatation of its capillaries rendering it a better con- 
ductor than it would naturally be in the dry state. In a healthy 
individual using an electro-motive force of from ten to fifteen 
volts the resistance amounts to from four to five thousand 
ohms, while in the course of this disease it measures from 
three to six hundred ohms, and only increases when the patient 



GRAVES' DISEASE. 



521 



improves. Eulenburg has shown that the presence of this 
symptom may be of great value for the diagnosis, but its ab- 
sence proves nothing (Centralblatt f. klin. Med., 1890, 1). 

Various nervous disturbances often accompany Graves' 
disease, among which we should first mention a peculiar para- 
paresis of the legs (effondement des jambes), a giving way of 
the legs, as it is called by the English authors, a condition 
which is associated with a flabbiness of the muscles and a 
diminution or loss of the patellar reflexes (Charcot). Eulen- 
burg regards the symptoms as a manifestation of hysteria, and 
as comparable to astasia-abasia (Neurol. Centralbl., 1890, 23). 
The digestive tract may be implicated, and we may have a 
well-marked intestinal atony (Federn, Wiener Klinik, 1891, 
Marz). Occasionally copious vomiting of watery bile occurs, 
and this symptom may be of such persistency as to seriously re- 
duce the strength of the patient. Vertigo, buzzing in the ears, 
sleeplessness, occasional transient dyspnoea, have also been ob- 
served. Falling out of the hair of the head and eyebrows is 
not rare, and I have seen a case of a peasant woman, thirty- 
eight years old, who, toward the end of the disease, when she 
was extremely emaciated owing to the persistent diarrhoea 
and vomiting, had become completely bald. As complications, 
bone disease (osteomalacia, Koppen, Deutsche Med.-Ztg., 1892, 
25, p. 296), chorea, epilepsy, psychoses (Schenk, Inaug.-Dis- 
sert., Berlin, 1890), e. g. mania, melancholia, neurasthenic in- 
sanity (Hirschel, Jahrb. f. Psychiatrie, 1893, 12), diabetes, tabes 
(Joffroy, Timotheeff, Inaug. -Dissert., Berlin, 1893), and Addi- 
son's disease (Oppenheim) have been observed. 

Course. — We should keep in mind that remissions may 
occur during the course of the disease, and may last even for 
months or years before further deterioration leading to death 
takes place. For the prognosis a knowledge of the fact that 
such remissions can occur is of great importance. Cases which 
pursue a rapid course from the beginning are exceptional. 
The onset of the disease may be either brusque or quite 
gradual. In the first case twelve to fourteen hours are suffi- 
cient time for the development of the three cardinal symp- 
toms ; in the latter these appear gradually — first the palpitation, 
then the swelling of the neck, and finally the protrusion of the 
eyeballs. 

Of great interest, because relatively frequently met with, 
are the cases in which the disease does not reach its full devel- 



522 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

opment : only certain symptoms are well marked, while others 
may be hardly perceptible or even absent. Trousseau calls 
these instances "formes frustes " {fruste — abortive), and attrib- 
utes much importance to them. P. Marie has subjected them 
to a careful study in his excellent monograph (cf. lit.), and has 
shown that the goitre as well as the exophthalmos may be 
wanting, in which case we shall only find the tachycardia, very 
often accompanied by a symptom to which he has given special 
attention — namely, the tremor. This tremor shows a great 
regularity of rhythm, and consists of about eight or nine 
oscillations in a second. Ernst Cramer, in his observations 
made in my wards, was generally able to confirm Marie's 
results. (Ueber das Wesen des Zitterns. Inaug.-Dissert., 
Breslau, 1886.) 

Prognosis. — The prognosis seems to be especially unfavor- 
able in man and in old age. Youth is by no means exempt 
from the disease, since Ehrlich (Inaug.-Dissert., Berlin, 1890), 
Kronthal (Berlin, klin. Wochenschr., 1893, 27), and others have 
reported cases of Graves' disease at the ages of ten, twelve, 
and thirteen. Female patients have, on the whole, a better 
chance for recovery, especially if they become pregnant 
(Charcot). 

-^Etiology. — We are not yet able to say anything definite 
about the aetiology of the disease, although it seems fairly cer- 
tain that heredity and an alteration in the thyroid gland have 
to be regarded as indispensable for the development of the 
disease ; all other factors, such as emotions, bodily overexer- 
tion (mountain climbing), cold, other diseases (e. g., influenza) 
are certainly less important and are probably never capable by 
themselves of producing the disease. 

Pathological Anatomy. — With regard to the anatomical 
changes it must be stated that the thyroid gland always shows 
a peculiar hyperplasia which differs from the ordinary goitre 
(William S. Greenfield, Brit. Med. Journ., 1893, December 9th). 
The vascular development was never found to be very striking ; 
microscopically an enormous increase in the secreting tissue 
was observed. According to Greenfield, this hyperplasia may 
exist for years before any symptoms of Graves' disease make 
their appearance. The changes which have been found in the 
sympathetic nerve of the neck and its ganglia are not constant, 
and although enlargement and thickening of the ganglia and 
of the nerve have often been noted, we can not draw any con- 



GRAVES' DISEASE. 



523 



elusions from these results, especially since several cases are on 
record in which the sympathetic was perfectly normal. 

In view of these results the disease must be regarded as 
due to a supersecretion of the product of the thyroid gland 
which has a toxic action. This assumption has received strong- 
support from the experiments of Greenfield. By giving dried 
thyroid extract to healthy individuals he produced tachycardia, 
irritability, irregular elevations of temperature, and a tendency 
to perspiration. George R. Murray (Lancet, 1893, ii, 20, No- 
vember nth) also favors this view, and Joffroy looks upon 
Graves' disease as a direct affection of the thyroid gland 
(Progr. med., 1893, 2, s., xviii). 

Treatment. — The most important part of the treatment 
consists in the (total or partial) removal of the thyroid gland, 
though there is by no means a general agreement in regard to 
this point, and it is still doubted by some whether a complete 
cure ever follows surgical interference. It seems certain, how- 
ever, that operation at any rate gives relatively the most favor- 
able results. It often happens that only certain symptoms 
are removed by such procedures. Thus, I have seen a case in 
which half a year after the operation the exophthalmos still 
persisted, while the subjective symptoms, especially the very 
disagreeable tachycardia, had completely disappeared. In 
every grave case of exophthalmic goitre, therefore, the ques- 
tion of operative interference should be carefully considered. 

Hack, Hoppmann,and others, have reported cases in which 
destruction of the swollen erectile tissue of the nose by the 
galvano-cauter}' brought about an improvement in some of the 
symptoms ; thus the exophthalmos at once disappeared on the 
side of the operation. In view of such cases, a rhinoscopic 
examination is always indicated. In other respects Graves' 
disease is treated as all other general diseases of the nervous 
system or of the entire organism. Cold-water treatment has 
been warmly recommended, be it in the form of wet packs or 
of prolonged douches ; patients in good circumstances should 
be sent every }^ear to a hydrotherapeutic establishment, since 
such courses are frequently followed by an appreciable though 
perhaps a not very marked improvement. Another procedure 
which deserves attention is the galvanization of the neck. 
The cathode is placed over the angle of the lower jaw, while 
the anode is applied over the lower cervical vertebras (on the 
opposite side) ; the current should be weak, and only applied 



524 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

for a minute to a minute and a half at a time ; often the symp- 
toms diminish steadily after ten or fifteen seances, usually after 
twenty or thirty, an improvement follows which may last for 
years (Erb, Benedikt, Guttmann, Mor. Meyer, and others). 
Whether this result is to be attributed to an action upon the 
vagus or the sympathetic can not be decided, since both these 
nerves are influenced by the current in the galvanization of 
the neck. 

Internal remedies are of comparatively little value. The 
tincture of strophanthus (two to ten drops every six hours for 
four weeks), which has been recommended by Brower, often 
leaves us in the lurch, and belladonna and iron are only of value 
in those mild cases occurring in young female patients which 
are apt to show well-marked remissions. In these an improve- 
ment of longer or shorter duration may occur under various 
drugs, but since this also happens when none at all are given, 
we have no right to attribute it to any particular medication. 
The marked improvement which sometimes occurs during 
pregnancy has already been spoken of. 

LITERATURE. 
Basedow, v. Exophthalmus durch Hypertrophic des Zellgewebes in der Augen- 

hohle. Casper's Wochenschr. f. d. ges. Heilkunde., 1840, No. 13, p. 197; 

und No. 14, p. 220. 
Trousseau. Du goitre exophth. Union med., Nos. 142, 143, 145, 147 ; Gaz. 

hebdom., pp. 219, 267; Gaz. des hop., i860, Nos. 139, 142. 
Oppolzer. Ueber Basedow'sche Krankheit. Wiener med. Wochenschr., 1866, 

Nos. 48 u. 49. 
Chvostek. Weitere Beitrage zur Pathologie und Elektrotherapie der Basedow- 

'schen Krankheit. Wiener med. Presse, 1872, Nos. 23, 27, 32, 39, 41-46. 
Eulenburg et Guttmann. Die Pathologie des Sympathicus. Berlin, 1873, pp. 

32 et seq. 
Sattler in Grafe-Samisch' Handbuch der Augenheilkunde. Leipzig, Engelmann, 

1880, vi. 
Marie, P. Contribution a l'etude et au diagnostic des formes frustes de la 

maladie de Basedow. Aux Bureaux du Progres med., Paris, 1886. 
Sainte-Marie, M. Contribution a l'etude de la maladie de Basedow. Th£se de 

Paris, 1887. 
Vigouroux. Sur le traitement et sur quelques particularites cliniques de la 

maladie de Basedow. Progres med., 1887, No. 43. 
Eulenburg. Berliner klin. Wochenschr., 1889, 1. 
J. Russel-Reynolds. Lancet, 1890, 3481. 

Kiimmel. Deutsche med. Wochenschr., 1890, 20. (Removal of Goitre.) 
Lemke. Deutsche med. Wochenschr., 1891, 2. 
Mobius. Deutsche Zeitschr. f. Nervenhk., 1891, i, 5, 6. 
Dreesmann. Deutsche med. Wochenschr., 1892, 5. 



MYXCEDEMA. 



525 



Determayer. Deutsche Med.-Ztg., 1892, 103. 

Wette. Arch. f. klin. Chir., 1892, xliv, Heft 3. 

Mendel. Deutsche med. Wochenschr., 1892, 5. (Pathological Anatomy.) 

Marcus. Wesen und Behandlung des Morbus Basedowii. Deutsche Med.-Ztg., 

1893, 48. 
Joffroy. Nature et traitement du goitre ophthalm. Progres med., 1894, 10-13. 
Marie, P. Deutsche Med.-Ztg., 1894, 29, p. 335. 
Mannheim. Der Morbus Gravesii. Berlin, 1894, Hirschvvald. 
Mobius. Schmidt's Jahrb., ccxli, 2, p. 134, 1894. 

II. MYXCEDEMA (GULL AND ORD), CACHEXIE PACHYDERMIQUE 

(CHARCOT). 

Myxcedema should be considered immediately after Graves' 
disease, because it may be regarded clinically as well as ana- 
tomically as the direct opposite of the latter affection (Green- 
field). While in Graves' disease we have a hyperplasia of the 
tissue of the thyroid and a hypersecretion, we find in myxce- 
dema an atrophy and replacement of the secreting tissue by 
hard fibrous tissue — myxcedema atrophicum. Myxedematous 
symptoms have also been observed in cases in which the thy- 
roid gland had been removed by operation — myxcedema oper- 
ativum. The cause of the spontaneous degeneration is not 
known ; the fact that the disease has been known to occur a 
number of times after exposure to wet and cold is, of course, 
not sufficient to establish the ^etiological importance of these 
factors. 

The general swelling has been found to be not an oedema, 
but to depend upon the development of a mucin-containing 
myxomatous new formation ; in the skin, the connective tissue, 
in the saliva and the blood, mucin can be demonstrated in con- 
siderable amount. Kraepelin has observed an increase in the 
diameter of the red blood-corpuscles, as well as an increase in 
the specific gravity of the dry residue of the blood (Deutsches 
Arch. f. klin. Med., xlix, 6, p. 587). Symptomatically the dis- 
ease manifests itself by a peculiar swelling of the whole face, 
the skin, especially in the region of the eyelids and the cheeks, 
appearing cedematous. The lips are not completely closed, 
and the saliva dribbles from the corners of the mouth. Owing 
to the stumpy thick nose and half-opened eyes the face be- 
comes somewhat uncouth and common-looking, and, later, ex- 
pressionless and cretinlike (cf. Fig. 160). The patients, espe- 
cially if they are females, grow to look so much alike that they 
appear as if they all belonged to the same family. The color 



526 



DISEASES OF THE GENERAL NERVOUS SYSNEM. 



of the face is pale, the skin is waxlike, but does not pit on 
pressure. The cedema of the rest of the body has the same 
character as that of the face. The skin of the neck forms folds, 
the hands are thickened. On the hard wrinkled skin circum- 
scribed thickenings can be seen, hair and nails fall out, the 
teeth become carious, the secretions diminish and dry up. 
Amenorrhcea is common. The lungs, heart, and large vessels 
present no abnormities. On examination the urine is found 
to be negative, while the temperature is subnormal. Among 
the concomitant symptoms must be mentioned sensory and 
motor disturbances, uncertainty in the gait, and general lassi- 
tude. Such patients get easily fatigued, and their mental 
faculties deteriorate {Idiot ie myxcedemateuse, Fig. 161). 




Fig. 160. — Case of Myxcedema. (After Charcot.) 

The disease is not easily mistaken, but Lassar has called 
attention to the fact that certain erysipelatous swellings may 
give to the face an expression similar to that seen in myxce- 
dema. 

The modern treatment of myxcedema is very satisfactory. 
The principle is to replace the missing or degenerated thyroid 



MYXCEDEMA. 



527 



gland. This can be done either by implanting a gland into the 
peritoneal cavity (Horsley), by injecting thyroid juice (Murray), 
or by giving it by mouth (Wichmann). The best thyroid to 
be used for the purpose is that of the sheep. Burroughs, Wel- 
come & Co., in London, have made compressed tablets of 




Fig. 161. — " Idiotie Myxcedemateuse." 
(After Bourneville ; Arch, de Neur., 189c, xix, 56.) 

powdered thyroid, each one of which contains five grains of 
the substance. Wichmann has obtained excellent results from 
their administration (Deutsche med. Wochenschr., 1893,43). P. 
Marie has also spoken very favorably of the thyroid treatment 
(Deutsche Med.-Ztg., 1894, 29, p. 335). 



LITERATURE. 

Gull. On a Cretinoid State supervening in Adult Life in Women. Transac- 
tions of the Clin. Society, 1874, vii, p. 180. 

Ord. On Myxcedema. Med.-Chir. Transactions, 1878, Ixi, p. 57. 

Hammond. On Myxcedema with Special Reference to its Cerebral and Nerv- 
ous Symptoms. Neurol. Contributions, 1886, i, 3, p. 36. 



528 DISEASES OE THE GENERAL NERVOUS SYSTEM. 

Charcot. Gaz. des hop., 1881, 10. 

Saville. Case of Myxoedema in a Male. Brit. Med. Journ., December 3, 1887, 

p. 1 2 16. 
Paton. Glasgow Med. Journ., December, 1887. 
Reverdin. Contribution a l'etude du myxcedeme consecutif a l'extirpation totale 

ou partielle du corps thyroi'de. Revue med. de la Suisse romande, 1887, 

5,6. 
Zielewicz. Berliner klin. Wochenschr., 1887, 22. 
Munk. Untersuchungen iiber die Schilddriise. Sitzungsber. der konigl. preuss. 

Akad. d. Wissensch., 1888. 
Probnik. Die Folgen der Exstirpation der Schilddriise. Arch. f. experim. Path. 

u. Pharm., 1888, xxv, 2. 
Conclusions of the Myxoedema Committee. Brit. Med. Journ., June 2, 1888, p. 

1 1 62. 
Mosler. Ueber Myxodem. Virchow's Arch., cxiv, Heft 3. 
Cousot. Idiotie avec cachexie pachydermique. Bull, de la Soc. ment. de 

Belgique, 1881, 51. 
Manasse. Berliner klin. Wochenschr., 1887, 47. 
Horsley. Brit. Med. Journ., February 8, 1890. 
Bircher. Samml. klin. Vortr., 1890, No. 357. 
Buzdygan. Wiener klin. Wochenschr., 1891, 31. 
Murray. Brit. Med. Journ., October 8, 1891, and August 27, 1892. 
Howitz. Ugeskrift for Laeger., 1892, 7-9, p. 109. 
Laache. Deutsche med. Wochenschr., 1893, 11. 
Lundiz. Edinb. Med. Journ., 1893, xxxviii, p. 996. 
Kinnicutt. New York Med. Record, 1893, xliv, 15. 
Rehn. Ueber die Myxodemform des Kindesalters und die Erfolge der Behand- 

lung mit Schilddrusenextract. Verhandl. d. Congr. f. innere Med., 1893, 

xii, p. 224. 
Vermehren. Stoffwechseluntersuchungen nach Behandlung mit Glandula thy- 

reoidea an Individuen mit und ohne Myxodem. Deutsche med. Wochen- 
schr., 1893, xix, 43. 



SECOND GROUP. 

NEUROSES IN WHICH THE ENTIRE ORGANISM IS MORE OR 
LESS SEVERELY IMPLICATED. 



CHAPTER I. 

NEURASTHENIA NERVOUS PROSTRATION. 

Neurasthenia (a, privative ; crOevos, force) or nervous ex- 
haustion is an affection of the nervous system with which the 
general practitioner meets very frequently, and is one of those 
diseases which may give rise to a good deal of error in diag- 
nosis and prognosis. At the same time it makes the most 
boundless demands upon the forbearance of the physician and 
upon the patience of the sufferer. The disease is a child of the 
modern mode of living, of the desire to become rich as soon as 
possible, and we look for it in vain in the old text-books. Al- 
though it may in earlier times have occurred now and then, 
the neurologists had neither opportunity nor occasion enough 
to study it intimately. This has only become possible quite 
recentlv, and it is certainly a fact of significance that neuras- 
thenia has been "discovered" in that continent, the inhabit- 
ants of which have the reputation of working the quickest, 
of living at the highest pressure, and therefore of being — of 
course with exceptions — more nervous and aging sooner than 
those of the Old World, to wit, in America. Beard, to whom 
we owe so many excellent observations, so many splendid 
hints for therapeutics, described it first and gave it the name it 
bears. Whereas the disease prior to Beard's publication was 
unknown, it soon began to prevail in such a striking manner 
and to be diagnosticated so frequently that one is almost led 
to think that this diagnosis is often arrived at in cases where 
something else exists, some organic affection possibly more 
difficult to recognize. The disease in question is not organic 
and not associated with any demonstrable anatomical altera- 
tions. Nobody has ever succeeded in finding any character- 
istic anatomical changes in individuals who have suffered for 

34 529 



530 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

years from the most pronounced neurasthenic manifestations 
and then have died from some intercurrent disease. A large 
number of subjective complaints, many of which fit into other 
clinical pictures, make it intelligible why a diagnosis of neuras- 
thenia is often made, sometimes without any sufficient, careful 
consideration of all the factors which ought to be taken into 
account. It is comfortable and presumes nothing. Its possi- 
ble incorrectness can frequently not be demonstrated, and it 
therefore rapidly attained a great popularity among physicians. 
Symptoms. — The first traces of the disease develop very 
gradually and imperceptibly. Sometimes they assume more 
of a cerebral, sometimes more of a spinal character, so that it 
has been thought justifiable to distinguish a spinal and a cere- 
bral neurasthenia (Encephalasthenia, Althaus, Deutsche med. 
Wochenschrift, 1894, 13). For the cases in which the symp- 
toms of derangement of digestion were most prominent the 
term gastric neurasthenia was coined, under which head 
we may possibly class certain of the so-called nervous dys- 
pepsias. Schott (Deutsche med. Wochenschrift, 1890, 34) 
has called attention to the neurasthenia cordis. In the ma- 
jority of cases the patients complain of getting easily and 
rapidly fatigued after the bodily exertion which is associated 
with their ordinary daily doings, whether at home or in their 
business, after walks, gymnastic exercise, etc. Things which 
they used to do without the least difficulty tire them greatly. 
In going distances which were formerly covered with ease they 
have to rest half way, and require more time to accomplish a 
given task. Not always are definite pains present. At times 
there are aches in the back and loins severe enough to be 
troublesome. Sensory disturbances, paresthesias, formication 
in the extremities, or numbness, are rarely absent. These feel- 
ings distress the patient and may make him fear he has tabes, 
and the idea that he is suffering from some spinal trouble is 
fostered by the circumstance that the sexual power is usually 
decidedly diminished, be it that the patient is unable to have 
connection as often as before, be it that the erection of the 
penis is incomplete or that no ejaculation of semen occurs. 
For married patients this weakness is a source of great distress 
and often is a very prominent symptom, and frequently it is 
this that finally decides them to consult a physician, a step which 
has been again and again deferred. The more we have to deal 
with neurasthenias the more frequently shall we make the ob- 



NE URA S THEN I A . r ^ r 

servation that the sexual functions are in the majority of cases 
in some way or other affected, and that the sexual neurasthenia 
particularly deserves the most careful attention of the physi- 
cian. To determine whether the complaints of a patient with 
regard to his disordered sexual functions depend upon organic 
disease or upon neurasthenia we have, besides a careful exam- 
ination of the genitals, to examine the urine. It is well known 
that the urine of neurasthenics not rarely presents a decided 
increase of urates, oxalates, and phosphates, and that not infre- 
quently spermatic fluid is passed during micturition or during 
defecation (Beard and Rockwell, cf. lit.). Where the patients 
complain, as they so commonly do, of impotence, we shall 
have to determine what form we are dealing with, and whether 
organic disease, more particularly atrophy of the testicles, is 
the underlying cause. Sometimes there exists only a decrease 
in the sexual desire, while the power remains the same ; some- 
times a decrease in the power and an increase in the desire, so 
that the ejaculation of semen occurs too early, sometimes be- 
fore the insertion of the penis. Again, both sexual desire and 
power diminish pari passu, or finally the potentia coeundi is nor- 
mal but there is absence of spermatozoa (" aspermatism "). 

All changes of this kind are noticed by the patient and 
their significance is ever exaggerated by a fervid imagination. 
Even in the cases in which in reality there is no disease and in 
which the impotence depends entirely upon psychical influ- 
ences, it makes itself disagreeably felt, and we must not forget 
that such a " psychical " impotence, in spite of all encouragement 
and all assurances on the part of the physician is sometimes 
more difficult to cure than one which depends upon organic 
disease of the sexual apparatus. Every abortive attempt at 
coitus exerts a depressing influence upon the patient for a con- 
siderable time and is quite liable to lead to a second failure, 
although all other conditions for the normal performance of 
the act may be favorable (Fiirbringer, cf. lit.). 

The disturbances of the cerebral functions which appear in 
the course of neurasthenia are very manifold. First, the pa- 
tient is down-hearted and worried and sees everything in the 
blackest colors, and, above all, despairs of recovery. He be- 
comes irritable and impatient, unsociable with his friends, and 
feared by his family. In his work he is less efficient. Duties 
which he previously performed without trouble seem hard to 
him and require twice or three times as long for their accom- 



532 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

plishment. Cases in which this is not a prominent feature, but 
where the working power remains unchanged, are met with, 
but are exceptions. The sleep is usually disturbed ; some- 
times a protracted insomnia adds to the trouble. Headache is 
not the rule, but the patient often complains of a disagreeable 
pressure in his head, which is accompanied with a slight feel- 
ing of dizziness. All functions share in the disorder, the appe- 
tite becomes bad, the bowels sluggish, the action of the heart 
feeble, and vaso-motor disturbances in the form of persistent, 
coldness of the hands and feet manifest themselves. The gen- 
eral condition of the patient is very pitiable in the higher 
grades of neurasthenia, and it is necessary for the physician 
to make a most careful examination so as not to go astray in 
the diagnosis. 

The objective examination, in contradistinction to what the 
manifold complaints of the patient might lead us to expect, re- 
veals strikingly little. Organic changes can not be demon- 
strated anywhere. Thoracic and abdominal organs are healthy ; 
nothing abnormal can be detected in the domain of the cranial 
nerves or in the fundi of the eyes. The condition of the pupils 
varies. Transient differences in their size — that is, unilateral 
dilatation, without, however, any abnormity in the pupillary 
reflex — is certainly met with. The dilatation may either al- 
ways be on the same side or change at times to the other eye. 
The phenomenon is usually marked when the general condition 
is bad, while it disappears if decided and lasting improvement 
is once established. The claim that lasting inequality of the 
pupils is always a sign of organic disease, as Beard thinks, must 
certainly be somewhat modified (Pelizaeus). I have myself 
seen differences in the pupils persist for eight or ten months 
and then disappear and the patient get well. 

The peripheral nerves as well as the tendon and skin re- 
flexes are normal. Tenderness over the vertebrae rarely is ab- 
sent, but is of no significance. 

Diagnosis. — When we have once sufficiently informed our- 
selves about these points the diagnosis will usually present but 
little difficulty. At first, it is true, we may be easily led astray 
and think of organic diseases of the brain, especially pro- 
gressive paralysis of the insane or a brain tumor, yet the fur- 
ther course of the disease will soon clear the matter up. The 
suspicion of tabes which may arise on account of the cerebral 
and particularly of the spinal symptoms, the disturbances of 



NE URA S THEN I A . r ^ . 

the sexual functions, and so forth, will be discarded, owing to 
the persistence of the patellar reflexes, the absence of actual 
bladder symptoms and pronounced sensory disturbances, an- 
aesthesias and hyperesthesias, as well as of actual motor weak- 
ness. Fibrillary twitchings, such as are observed in progress- 
ive muscular atrophy, may here also be met with, but they 
are seen rarely and their occurrence varies a good deal. From 
hysteria neurasthenia is distinguished by the fact that the con- 
stant change of the symptoms which is so characteristic of hys- 
teria, besides the circumscribed neuralgias, the contractures, 
the spasms, etc., is here not observed. Still, to make a diag- 
nosis, repeated and careful examinations are needed, to which 
the patients do not submit as willingly as hysterical men and 
women. 

^Etiology. — In every case in which hereditary influences 
can be excluded the prime cause of neurasthenia is unques- 
tionably to be looked for in an overtax of the nervous sys- 
tem. This is brought about in many ways, by excessive men- 
tal work or by habitual bodily overexertion. It may be at- 
tributable to repeated emotions or to sexual excesses. Under 
the latter head we may put masturbation, which is a wide- 
spread evil among the young of both sexes and the practice of 
which not only may begin very early, but may be continued 
much longer than the physician himself might suspect. One 
may say that there are but few neurasthenics who have not 
during their youth been addicted to this habit for a longer or 
shorter period of time. " Sexual perversion " (Spitzka) and 
the various kinds of " psychical masturbation " may also be- 
come of astiological significance. Even in married life, where 
the satisfaction of the sexual desire is otherwise well regulated, 
the coitus interruptus sive reservatus, which is practiced to 
avoid too great an increase in the family, may afford a cause 
for neurasthenia. In my experience very few men have been 
able to practice with impunity for years this coitus interruptus, 
and it is the bounden duty of the physician to inquire with 
much tact but still with perseverance into this question. 

In some cases the abuse of tobacco may lead to neuras- 
thenia, so that the latter has to be looked upon directly as a 
nicotine poisoning, and must, of course, be treated accordingly. 
Persons whose occupation necessitates work not only energetic, 
but also associated with the emotions (artists, students, finan- 
ciers, speculators, etc.), also those whose occupation entails at 



534 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



the same time bodily as well as mental strain, are all more or 
less neurasthenics. Not rarely repeated losses of a consid- 
erable quantity of blood produce neurasthenia by causing a 
general anaemia, yet we must definitely state that the neuras- 
thenia may occur very well in such cases without the anaemia. 
Traumatism also may cause neurasthenic conditions. About 
these, which are usually intermediate forms between this dis- 
ease and hysteria, we shall have to speak later, under the head 
of " traumatic neuroses." Finally, neurasthenia has been known 
to occur after infectious diseases, typhoid fever, cholera, vari- 
ola. In these cases the bad state of nutrition and the faulty 
condition of the blood have to be held responsible. 

Treatment. — The treatment of neurasthenia is one of the 
most troublesome tasks which the physician encounters. It is, 
of course, not sufficient to give the patient a prescription and 
let him go. We must frequently examine him, not only on our 
own account, but for his own sake as well, because he is com- 
forted by the attention and solicitude of the physician, although 
the examination itself is usually disagreeable to him. There are 
neurasthenics who are actually relieved by repeated examina- 
tions, although nothing is ever prescribed. They gain there- 
from the quieting conviction that somebody is looking out for 
them, and this gives them hope. But here also the direct 
psychical treatment, such as we usually find to be of value 
in hysteria, is of the greatest importance. The patient must 
again and again be encouraged and told that all his organs are 
healthy, that it is only a nervous overstrain which he is suffer- 
ing from, a deficit in his nerve capital which it is somewhat 
difficult to replace. To exert a mental influence upon the pa- 
tient in this manner time is necessary, and those physicians who 
can not afford sufficient time for the purpose should not take 
charge of a grave case of neurasthenia at all. 

If hypochondriacal notions are prominent features, so that 
the patient is beyond the reach of consoling and encouraging 
words, the question whether or not he should be removed to 
an institution must come under consideration. In addition to 
the fact that change of air and scene exerts in itself a favorable 
influence, it is advisable to place a neurasthenic after a certain 
time among different surroundings, so that he has to meet with 
different people and has something fresh to occupy his mind 
with, and care should be taken to keep him constantly under 
the guidance of a physician. As supplying such requirements 



NE URA S THEN I A. 



535 



sanitaria for nervous people, in which insane cases are not re- 
ceived, are to be highly recommended. Of course the pecu- 
niary condition of the family must, before deciding- upon this, 
be taken into account, since all establishments of this kind in 
which patients are well cared for are rather expensive. Some- 
times in the more favorable instances a stay of from four to six 
months is sufficient to bring about a very decided improve- 
ment, in which case even families who are not very well off 
should be able to afford the expense. 

There are especially two factors from which much is to be 
expected in the treatment of neurasthenia, and these are elec- 
tricity and hydrotherapy, particularly the cold-water treat- 
ment. With regard to the former it may well be stated that 
there is no other nervous affection in which its application is 
followed by such excellent results as here. Used at the proper 
time and in proper doses, so to speak, it is most beneficial. 
The method which is best employed and to which we give by 
far the preference is the so-called general faradization as rec- 
ommended by Beard and Rockwell, as well as the general gal- 
vanization. The results are especially striking if we make use 
of the brush, which, in Beard's method, is not only applied to 
the back — although it is kept here longest — but (with the ex- 
ception of the head) all over the body. Although the patient 
may complain of disagreeable and painful sensations for the 
five or eight minutes during which the sitting lasts, the after 
effects which soon follow are most gratifying. The patients 
feel invigorated and leave the physician with a sense of having 
gained a new lease of life. According to our experience the 
faradization as advised by Beard is superior in its action to the 
electrical baths, which are much more circumstantial and have 
not been as yet sufficiently studied. 

With reference to the cold-water treatment, to which we 
have repeatedly called attention in different places, we must in 
this more than in any other affection warn against overzeal- 
ousness and insist upon caution. Low temperatures are borne 
very badly by these nervous and irritable patients. They be- 
come excited and sleepless, and our aim is not only frustrated, 
but actually more harm than good is done. However, if we 
cautiously begin with a temperature of from 86° to 78 F., and 
confine our measures to gentle rubbings, affusions of short 
duration, cool hip baths, also of short duration, and avoid 
douches altogether, if care is taken at the same time to insure 



/ 



536 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

proper nourishment and exercise for the patient, the best form 
of which, perhaps, is a walk in the woods, the results are en- 
couraging and lasting, if neither physician nor client lose their 
patience too soon. A course of treatment of this kind can not, 
however, be compressed into the usual four weeks of a summer 
vacation, but to do any good six, eight, or ten weeks should be 
taken. Sometimes sea baths will be more useful than the 
simple cold-water treatment, but then also care must be taken 
in their selection. For the excitable and nervous who suffer 
from insomnia the places on the Baltic will on the whole be 
preferable to those on the North Sea, while the latter are 
especially adapted for very prostrated patients and individuals 
suffering from cerebral anaemia. 

A long stay in pure mountain air, at a not too high altitude 
and where the barometer is not too low, is usually beneficial 
to neurasthenics. Daily systematic, but not forced, tramps in 
the mountains, continued for weeks, do more good sometimes 
than all the medicines of the pharmacopoeia taken during the 
long winter. The internal medicines are anyhow of not much 
avail in the treatment of neurasthenia. Iron, quinine, arsenic, 
the stomachics, all will disappoint us ; all will sometimes ac- 
complish nothing ; they rather tend to derange the digestion, 
and with this take away the last remnant of the patient's 
courage. The only drugs necessary will be such as are re- 
quired for the proper regulation of the bowels. 

Among the above-described symptoms there are two the 
treatment of which deserve special mention — first, the sleep- 
lessness ; secondly, the impotence. About the former nothing 
needs to be added to what has been said on page l^%r^ To 
meet the latter much is to be expected — if, of course, organic 
disease, spermatorrhoea, and the like, have been excluded — from 
the local application of electricity. A large electrode, the 
anode, is placed over the lumbar cord, while the cathode is 
moved from the external inguinal ring down along the sper- 
matic cord or applied without being shifted (Erb). With this 
may be combined the application of the faradic brush over 
the whole genital region. One electrode, the cathode, may 
also be placed in the rectum, the other upon the sacrum or 
perineum (Mobius). Finally, a bladder electrode, which has 
the shape of a catheter, and which is insulated up to its metal- 
lic tip, may be introduced into the urethra as far as the fossa 
navicularis, while the anode is applied over the lumbar cord, 



NE URA S THEN I A . 



537 



and at the negative pole the current is made and broken sev- 
eral times. From this method, which has been recommended 
more especially for paralysis of the bladder and incontinence 
of urine, we have repeatedly seen good effects in the treatment 
of impotence. 

The feeding system of Weir Mitchell, which has also been 
recommended in neurasthenia, we shall discuss in the chapter 
on hysteria. 

LITERATURE. 

Beard. On Neurasthenia. New York, 1880. 

Eisenlohr. Deutsche med. Wochenschr., 1884, x, 21. (Differential Diagnosis 
between Tabes and Neurasthenia.) 

Beard und Rockwell. Die sexuelle Neurasthenic Wien, 1885. 

Mobius. Die Nervositat. Leipzig, 1885, 2. Aufl. 

Thayer. Neurasthenia. Phil. Med. and Surg. Report., 1886, liv, 17, 18. 

Berdt Hovell. On some Conditions of Neurasthenia. London, 1886, Churchill. 

Averbeck. Die acute Neurasthenic Deutsche Med.-Ztg., 1886, vii, 30, 31, 

Langstein, H. Die Neurasthenic Wien, 1886. 

Mitchell, S. Weir. An Essay on the Treatment of Certain Forms of Neuras- 
thenia and Hysteria. Phila., Lippincott, 1885. 

Uherek. Die functionellen Neurosen beim weiblichen GeschLcht und ihre 
Beziehungen zu den Sexualleiden. Berlin, Neuwied, 1886. 

Clark. Some Observations concerning what is called Neurasthenia. Lancet, 
January 1, 1886, i. 

Krafft-Ebing, v. Ueber Neurasthenia sexualis beim Mann. Wiener med. 
Presse, 1887, xxviii, 5, 6. 

v. Ziemssen. Die Neurasthenie und ihre Behandlung. Leipzig, Vogel, 1887. 

Hanc. Ein seltener Fall sexueller Neurasthenie. Wiener med. Klinik, x, 5. 

Pippinskold. On neurastheniens forekomst bland kroppsarbetarc Finska 
lakaresallsk. handl., 1887, xxix, 11. 

Burkart. Berliner klin. Wochenschr., 1887, xxiv, 45. (Recommends Weir Mitch- 
ell's treatment.) 

Mathieu. Neurasthenie et hysterie combinees. Progr. med., 1888, xvi, 30. 

Lemoinc Pathogenie et traitement de la neurasthenie. Ann. med. psych., 
Septembre, 1888, 7me ser., viii. 

Webber. A Study of Arterial Tension in Neurasthenia. Boston Med. and 
Surg. Journ., May, 1888, cxviii, 18. 

Fiirbringer. Zur Kenntniss der Impotentia generandi. Deutsche med. Wo- 
chenschr., 1888, xxv, 28. 

Wagner. Zur Begriffsbestimmung und Therapie der Neurasthenie. Schvveizer 
Correspondenzbl., 1888, xviii, 9. 

Pelizaeus. Zur Differentialdiagnose der Neurasthenic Deutsche Med.-Ztg., 
1889, 27, 28. 

Lowenfeld. Die moderne Behandlung der Nervenschwache (Neurasthenie), der 
Hysterie und verwandter Leiden. Wiesbaden, Bergmann, 1889, 2. Aufl. 

Pitres-Bitot. De la Neurasthenie et de l'hystero-neurasthenie traumatique. 
Progres med., 1890, 49. 



538 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Bouyer. La neurasthenie (epuisement nerveux). Paris, Bailliere, 1890. 
Bottey. Hydrotherapie et Neurasthenie. Revue d'hygiene therap., Fevr. 

1892. 
Jacobs. Gen. Tijd. voor Nederld. Indie., 1892, xxxii, 5. 
Sollier. Sur une forme circulaire de la neurasthenie. Revue de med.. 1893, 

xiii, 12. 
Muller (Alexanderbad). Handbuch der Neurasthenie. Leipzig, Vogel, 1893. 

(Indispensable for special studies.) 
Kothe. Wesen und Behandlung der Neurasthenie. Weimar, 1894. 



CHAPTER II. 

HYSTERIA. 

Hysteria has this in common with neurasthenia, that it 
does not depend upon any demonstrable anatomical lesions of 
the nervous system, but it differs from it in the fact that for 
its development a certain predisposition on the part of the pa- 
tient is absolutely necessary. Although we are not as yet in a 
position to say of what nature this predisposition is, we must 
assume that the whole nervous system of a hysterical patient, 
central as well as peripheral, is in some points, which we are 
still unable to determine, different from that of healthy indi- 
viduals. The greater extent to which these persons observe 
themselves (Oppenheim), the increased impressionability, the 
hypersesthesia of the central nervous organs, the increased 
sensitiveness of the peripheral nervous system, the diminished 
energy with which influences coming from outside as well as 
from within are met, the lower general power of resistance 
and self-control, these are on the whole the traits which charac- 
terize hysterical persons, and explain why the symptoms are 
so manifold and change so rapidly, and why in no other disease 
of the nervous system can be found a train of manifestations so 
diverse and so numerous. 

Only by unwearied, long-continued study has it been possi- 
ble to show that even for the apparently arbitrary appearance 
of the different symptoms there exist certain laws. In a man- 
ner which none before or after have been able to rival, hysteria 
has been studied by Charcot and his pupils, to whom we owe 
the most interesting observations and investigations of the past 
two decades. 

LITERATURE. 

Hasse. Krankheiten des Nervensystems. In Virchow's Handbuch der speciellen 
Pathologic und Therapie. Erlangen, 1869. (Contains the older literature.) 

Briquet. Traite clinique et therapeutique de l'Hysterie. Paris, 1859. (Original 
article, upon which all the studies on hysteria by the French authors are 
based.) 

539 



540 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Charcot. Klinische Vortrage iiber Krankheiten des Nervensystems. German 
translation by Fetzer, Stuttgart, 1874. 

Charcot. Neue Untersuchungen iiber die Krankheiten des Nervensystems, 
besonders iiber Hysteric German translation by Freud. Wien und Leip- 
zig, 1886. 

Freud. Beitrage zur Casuistik der Hysteric Wiener med. Wochenschr., 1886, 

49. 50- 
Mobius. Ueber den Begriffder Hysteric Centralbl. f. Nervenhk., 1888, xi, 3. 
Thermes. Traite elementaire d'Hygiene et de Therapie de l'Hysteric Paris, 

1889: 
Charcot. Lecons du Mardi a la Salpetrierc Paris, 1889. (Policlinique, 1887— 

1888.) 
Gilles de la Tourette. Traite clinique et therapeutique de l'hysterie, d'apres 

l'enseignement de la Salpetrierc Paris, 1891. 
Gilles de la Tourette. Die Hysterie nach den Lehren der Salpetrierc German 

translation by Karl Grubc Wien, Deuticke, 1893. 
Breuer und Freud. Ueber den psychischen Mechanismus hysterischer Pha- 

nomene. Neurol. Centralbl., 1893, xii, 1, 2. 

Symptoms. — For the sake of simplicity we shall divide, in 
our description of the disease, the symptoms into cerebral, spinal, 
and mixed — that is, pertaining to the entire nervous system. 

The cerebral may again be subdivided into psychical and 
somatic symptoms. The disposition of the patients is excitable, 
anxious, often changeable, sometimes passing from the depths 
of gloominess to the most exalted hilarity. The tendency to 
speak of nothing else than of their own woes, the constant at- 
tempt to greatly exaggerate these, and to excite sympathy in 
their friends and physicians, the thoughtless demands which 
they expect to be satisfied at a moment's notice, and the incon- 
siderate outbreaks of anger if this is not done — all these are 
characteristic features of the disease with which we meet, not 
in all indeed, but at any rate in a large majority of cases. The 
tendency to get easily frightened is very common, and during 
a state of the highest psychical excitement hallucinations may 
temporarily exist. In pure cases of hysteria, however, we 
need never be afraid that these will persist long or lead to an} 7 
serious outbreak on the part of the patient. Exceptionally an 
instance of " hysterical sleep " comes under our notice, into 
which the patient has fallen after certain prodromal symptoms 
have existed for several hours. The peculiarities of this curi- 
ous condition, the " lethargie hysterique," which may last for 
many days, the condition of the organs of circulation and di- 
gestion, the characteristic signs by which the hysterical sleep 
may be discriminated from other states of coma, have recently 



HYSTERIA. 



541 



been described by Gilles de la Tourette in a careful monograph 
(Arch, de Neurol., 1888, 43, 44), and lately by Loewenfeld 
(Arch. f. Psych., xxii and xxiii). The paroxysmal appearance 
of a marked tendency to sleep (narcolepsy) has been studied 
by Bohm and Dehio (cf. lit.). 

Among the cranial nerves there is not a single one which 
may not at one time or another in the course of hysteria pre- 
sent symptoms of paralysis or irritation. More than the 
others the nerves of special sense are interesting for their 
anaesthesias and hyperaestheias. The nerves of smell and 
hearing are those most frequently affected, and both functions 
may be so much impaired that the patient can smell and hear 
nothing. They may, on the other hand, become so acute that, 
if we may believe her own statements, she is able to distin- 
guish any one from a number of perfumes, or to single out an 
individual by the sound of his voice amid the hubbub of a 
crowd, or, again, to recognize people far off by their step, and 
so forth. These and similar faculties have in Mesmer's time 
already been spoken of a great deal, and have given rise to 
much deception and trickery. The opticus is also not rarely 
affected. Besides the cases where hysterical patients suddenly 
become blind in one or both eyes without there being any 
changes in the disk, there are instances of decrease in the 
acuteness of vision, contraction of the field of vision, or com- 
plete or partial loss of color sense. When the last-named con- 
dition occurs the perception of blue and yellow is retained 
longest, while that of violet and green disappears much ear- 
lier. We must of course expect numerous variations and com- 
binations. I have seen in the same individual hysterical 
changes in the one eye and tabetic changes in the other. The 
ocular muscles rarely participate in the disease ; hysterical 
paralysis of them is exceptional, as is also the occurrence of 
hysterical nystagmus, on which subject I have expressed my 
opinion elsewhere (cf. lit.). 

Among the other nerves of special sense that of taste may 
occasionally present alterations. The patients lose their taste 
either completely or only for certain substances (sour, salty), 
or there may exist such a perversion of this faculty that every- 
thing tastes nauseous and disgusting, or that everything tastes 
of salt or of vinegar, and so forth. Actual hallucinations of the 
sense of taste, although not so frequent as hallucinations of the 
sense of smell, are not unheard of. 



542 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



The trigeminus is generally implicated. Faceache and 
headache, among others the kind which is confined to a small 
spot and is known as clavus, are comparatively frequent. The 
scalp is sometimes so markedly tender that the patients can 
not stand the slightest pressure, not even the touch of the 
comb, and in order to avoid the pain they abstain from all 
care and proper attention to the hair. The pain in the head 
may also be confined to one side, and resemble in every detail 
that of hemicrania. 

What needs to be said about the facial nerve in this connec- 
tion has already been treated of in Chapter V, Part II. Tic 
convulsif, as well as facial paralysis, may be hysterical in na- 
ture ; however, we must not forget that facial spasm and hys- 
teria may well coexist, and that a tic convulsif occurring in the 
course of hysteria is not necessarily of hysterical origin. The 
determination of this question is less important for the diag- 
nosis than for the prognosis. The outlook in non-hysterical 
tic is very bad, in the hysterical variety relatively favorable 
(Guinon, Revue de med., Juin, 1887). Of much interest are 
the many forms of vagus neuroses which we meet with in the 
course of hysteria ; they may affect, in the manner described in 
Chapter VIII of Part II, the organs of respiration, circulation, 
and digestion. Among the first, not only the larynx but the 
lungs also are sometimes attacked. The laryngeal muscles 
become the seat of violent spasm, " hysterical spasm of the 
glottis," during which the patient is afraid she is choking. In 
exceptional cases patients have died in such attacks (Leo, 
Deutsche med. Wochenschr., 1893, 34). The functions of the 
vocal cords may become so much interfered with that the 
patient is only able to make herself understood in whispers ; 
to speak out loud is impossible (" hysterical aphonia "). The 
laryngoscopical examination reveals nothing abnormal, with 
the exception of some anaesthesia of the mucous membrane of 
the fauces, which greatly facilitates the examination (cf. page 
113). Peculiar disturbances in speech — for example, a stutter- 
ing, which, in contradistinction to the ordinary type, comes on 
acutelv — have been frequently observed and carefully studied. 
For the recognition of this symptom and its differentiation from 
ordinary stuttering verbal suggestion may be used (cf. the chap- 
ter on Hypnotism). The respiratory muscles may be affected in a 
peculiar and very striking manner ; the acceleration in the num- 
ber of respirations may attain such a degree that, instead of 



HYSTERIA, 



543 



fifteen or sixteen respirations a minute, we may count from 
eighty to one hundred. On the other hand, they may be dimin- 
ished in frequency, and the patient breathe from eight to ten 
times a minute, but in a labored way, showing signs of a regu- 
lar dyspnoea, not infrequently with audible wheezing in inspi- 
ration and expiration (" hysterical asthma"). A dry and bark- 
ing cough, which is distressing not only to the patient but also 
to all who surround her, is sometimes observed, and paroxysms 
of yawning, sobbing, laughing, or crying (" hysterical laughing 
or crying fits ") may persist for hours. 

Sometimes following aphonia, sometimes occurring abrupt- 
ly and unexpectedly without it, in rare instances a complete 
dumbness sets in ; the patient has either actually lost the con- 
trol of her speaking apparatus or will not make use of it ; in a 
word, she is completely mute, and no amount of admonitions, 
entreaties, or threats can succeed in eliciting a single word. 
This condition of "mutismus hystericus'' may be of variable 
duration. In one instance which came under my notice the 
patient maintained silence from the 5th of September to the 
28th of April of the following year. She found her voice again 
at once on hearing of the unexpected death of her mother. In 
this connection the articles of Natier, Huysmann, and of Kay- 
ser (Therap. Monatsh., October, 1893, vii, p. 500), who recom- 
mends autolaryngoscopy as a useful means of treating this 
symptom, may be referred to. 

The circulatorv organs more especially the heart, take 
relatively the smallest share in the disease. Hysterical tachy- 
cardia may occur, but it is rare and never well marked ; 
even in the apparently severest attacks, which we shall de- 
scribe later, the pulse is quiet. To stenocardia we have re- 
ferred on page 123. 

Cases of so-called " aortic hysteria," a condition which has 
been described by Post, of New York (Med. Rec, 1891, 16), 
and which is characterized by relaxation of the aortic walls in 
consequence of diminution of the vascular tonus, simulating 
a tumor, are of a very rare occurrence. 

The digestive tract and the muscles pertaining to it — which, 
just as the pharyngeal muscles, are innervated at least partly 
by the glossopharyngeal and not by the vagus alone — may be 
the seat of various hysterical manifestations. The muscles of 
the pharynx may present symptoms of paralysis or of irrita- 
tion. In the former case deglutition is much interfered with, 



544 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

and may, indeed, be impossible (" hysterical deglutition paral- 
ysis "). 

A peculiar affection of the muscles of the oesophagus, 
which are supplied by the vagus, consists in a spasmodic con- 
traction which gives rise to a very vivid sensation of a ball 
rising up from the region of the stomach and sticking in the 
throat. This " globus hystericus" is so frequently met with in 
hysteria and is usually so well marked, that it has been looked 
upon as pathognomonic for the disease. 

The musculature of the stomach and the intestines is liable 
to disturbances. According to most authors, paralysis of these 
muscles produces a distention of the bowels and of the whole 
abdomen which may be simply enormous (" meteorismus hys- 
tericus " ) ; this is sometimes associated with colicky pains. A 
certain amount of the air, which frequently collects in large 
quantities in the bowels, escapes through the mouth with a 
loud, sobbing, gurgling noise (singultus, ructus hystericus). 
Talma (Weekblad van het Nederl. Tijdschr. voor Geneesk., 
1886, 9) claims that the cause of hysterical tympanites is to be 
sought in a spasm of the diaphragm. As evidence in favor of 
his view he argues that under chloroform narcosis the disten- 
tion will disappear without the emission of gas ; and, secondly, 
that the position of the diaphragm is abnormally low. 

Vomiting is one of the most frequent occurrences in hys- 
teria ; sometimes it is very profuse and may persist for hours ; 
it may be so intractable as to weaken the patient considerably ; 
on the other hand, slight vomiting may occur daily for weeks 
without affecting the patient's strength. Usually watery 
masses are thrown up which bear no proportion to the quan- 
tity of food ingested. In one of my cases the amount vomited 
was eight or ten times as large as that taken in. 

Affections of the accessorius are not rarely seen in the form 
of spasmodic torticollis, while affections of the hypoglossus are 
very exceptional. 

LITERATURE. 

Parinaud. Annal. d'Oculiste, 1886, xcvi, 1, 2. (Anaesthesia of the Retina.) 
Brissaud et Marie. Progres med., 1886, xv, 5, 7. (Deviation faciale dans 

l'hemipl. hysterique.) 
Guinon. Revue de med., 1887, vii, 6. (Tic convulsif.) 
Huet. Hysterical Facial Paresis. Nederl. Weekblad, 1887, ii, 22. 
Borel. Annal. d'Oculiste, 1887, xcviii, 5, 6. (Hysterical Affections of the Eye 

Muscles, etc.) 
Schlesinger. Wiener med. Blatter, 1888, xi, 3. 



HYSTERIA. 



545 



Peek. New York Med. Rec, March, 1888, xxxiii. (Hysterical Coma.) 

Fere. Migraine ophthalmique hysterique. Arch, de Neurol., 1890, 60. 

Remak, E. Zur Semiotik der hysterischen Deviation der Zunge und des Ge- 
sichtes. Berliner klin. Wochenschr., 1892,40. 

Leber. Periphere Sehnervenaffectionen bei Hysteric Deutsche med. Wochen- 
schr., 1892, 33. 

Hitzig. Schlafattacken und hypnotische Suggestion. Berliner klin. Wochen- 
schr., 1892, 38. 

Przeborski. Monatschr. f. Ohrenhk., 1893, 11. 

Fere, Ch. Semaine med., 1883, xiii, 50. 

Blocq. Gaz. des hop., 1893, 135. 

Janet, P. Etat mental des Hysteriques. Paris, Rueff, 1893. 

Knies. Die einseitigen centralen Sehstbrungen und ihre Beziehung zur Hysterie. 
Neurol. Centralbl., 1893, 17. 

Bbhm. Ueber Narkolepsie. Inaug.-Dissert., Berlin, 1893. 

Dehio. Pathologische Schlafzustande. Petersburger med. Wochenschr., 1893, 
22. 

Hysterical Speech-disturlances. 

Peltesohn. Berliner klin. Wochenschr., 1890, 30. 

Boedeker. Charite-Annalen, 1890, xv, p. 373. 

Rosenbach, O. Ueber functionelle Lahmung der sprachlichen Lautgebung. 

Deutsche med. Wochenschr., 1890, 46. 
Ballet et' Tissier. Du begaiement hysterique. Arch, de Neurol., Juillet, 1890, 

No. 58. 
Kramer. Ueber hysterisches Stottern. Prager med. Wochenschr., 1891, xiv. 
Chervin. Apropos du begaiement hystenque. Arch.de Neurol., Mai, 1891, 

No. 63. 
Higier. Ueber hysterisches Stottern. Berliner klin. Wochenschr., 1893, 34. 
Seiffert. Die Behandlung der hysterischen Aphonic Ibid., 1893, 44. 
Zoeller. Fall von hysterischer Stummheit von zweijahriger Dauer. Neurol. 

Centralbl., 1894, 2. 

One of the most remarkable cerebral affections which may 
occur in the course of hysteria is an apoplectiform attack with 
consequent hemiplegia, which in many instances is associated 
with complete hemianassthesia. This hemiplegia may develop 
with symptoms similar to those of the form following arterial 
disease, and, as, we have already pointed out above, it may be 
extremely difficult to distinguish a hysterical hemiplegia from 
one due to organic disease. This is especially the case if there 
are no other hysterical symptoms to aid us. If the unilateral 
spasm of the muscles of the cheek, described by Charcot, and 
before him by Brodie (1880), which is said to be characteristic 
of hysterical hemiplegia, be present, the diagnosis is easier. 
All the symptoms associated with a cerebral hemiplegia — for 
instance, tremor, the associated movements, even atrophy of 
the muscles of the side affected — may accompany the hyster- 

35 



54^ 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



ical variety. The opinion formerly prevalent, that wherever 
there exists atrophy this must needs depend upon an organic 
lesion in the brain, spinal cord, or the nerves, has been proved 
to be erroneous. The hysterical atrophy may not differ from 
that due to organic disease ; it may develop comparatively 
rapidly, may remain for a long time, and disappear again just 
as rapidly when motion returns. Fibrillary twitchings in the 
atrophic muscles and reaction of degeneration are absent. 




Fig. 162. — Patient shown in Fig. 163, three months previous to the time when the picture 
of Fig. 163 was taken (personal observation). 

Whether the large ganglionic cells in the anterior horns have 
anything to do with the occurrence of atrophy, and, if so, 
what is the nature of the influence, we do not know. 

I will here mention only one of the cases of hysterical 
atrophy which have come to my notice and which is quite 
unique, owing to the intensity and the rapidity with which 
an atrophy of the entire muscular system developed. The 
clinical history of the case, of which two pictures (Figs. 162 



HYSTERIA. 



547 



and 163) are here given, will be found in an article by me in 
the Deutsche med. Wochenschrift. The time which elapsed 
between the taking of the two pictures was about three months. 




Fig. 163. — Fatient with muscular atrophy, shown in Fig. 162 (personal observation). 



LITERATURE. 

Babinski. Arch, de Neurol., Juillet, 1886, vii. (Atrophy in Hysterical Paraly- 
sis.) 

Chauffard. Gaz. hebd., 2. s., 1886, xxiii, 21. (Atrophy of the Left Upper Ex- 
tremity, Hysterical Monoplegia, as a Result of Trauma in a Young Man.) 

Massalongo. L'atrofia muscolare nelle paralisi isteriche. Napoli, Detken, 1886. 

Debove. De l'apoplexie hysterique. Arch, gener. de med., 1886, No. 34. 

Voisin. Arch, de Neurol., 1886, xii, p. 202. (Hysterical Monoplegia of Six 
Months' Standing, cured immediately by Suggestion.) 

Oserezkovvski. Zur Diagnose der hyster. Hemiplegie. Centralbl. f. Nervenheilk., 
1887, x, 6. 

Martinencq. Cas d'apoplexie hyst. avec autopsie. Ann. med. -psych., 7 Mars, 
1887. 

Brissaud. Arch, de phys. norm, et pathol., 1887, 3. (Hysterical Hemiplegia 
with Atrophy.) 



548 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Achard. De l'apoplexie hysterique. Arch, gener. de med., Janv., 1887. 
Moravsik. Centralbl. f. Nervenheilk., 1888, xi, 20. (Hysterical Symptoms in 

Brain Syphilis.) 
Souques. Hemiplegie hysterique (chez un saturnin). Gaz. de Paris, 1889, 2. 

Among the spinal symptoms of hysteria, motor and sensory 
paralyses play the most important role. With hysterical pa- 
tients we can not feel certain for a single day or hour that 
some sort of paralysis will not occur, for it is characteristic, 
we may say pathognomonic, of hysterical paralyses that they 
appear quite suddenly, and happily often disappear as quickly, 
it may even be after persisting for months and years. There 
is no characteristic distribution of the hysterical motor dis- 
turbances ; they may take in only one extremity, or may ex- 
tend to both legs or both arms, so that these are perfectly 
useless. Examination shows that the paralyses are usually of 
a flaccid type. We may frequently make the observation that 
the patients are not completely robbed of the use of the af- 
fected limbs, but that they have lost the will to use them. 
Especially is this apparent when they are asked to perform 
co-ordinated movements. A patient, though able to move the 
right arm, may assert that she is unable to write ; though she 
is able to move her legs, any attempt at walking is an utter 
failure ; on rising, her legs give way under her, and she simply 
is unable to keep herself on her feet. The inability to stand 
and walk, which is sometimes found in cases of hysteria, was 
first studied by Paul Blocq, and was termed by him astasia- 
abasia (Arch, de Neurol., Janvier, 1888, xv, No. 43); when the 
patient is in a recumbent position the sensation, the muscular 
power, and the co-ordination of the legs present no abnormity. 
Mobius, who among others has studied this condition care- 
fully, has called attention to the fact that the patient knows 
nothing of its origin ; that it develops through (unconscious) 
auto-suggestion, but that the subsequent amnesia hides from the 
patient the true origin of this suggested alteration. " The 
suggested idea does not become a part of consciousness in the 
waking state ; it does not become a motive for the will, as do, 
for example, fixed ideas, but acts subconsciously " (Mobius). 
Charcot, in his Legons du Mardi, has distinguished a paralytic 
and an ataxic form of hysterical abasia (Lecon du 5 Mars, 1889). 
A critical review by Mobius of all the cases published up to 
1890 will be found in Schmidt's Jahrbucher, 1890, ccxxvii, p. 25. 

Symptoms of motor irritation — for instance, isolated raus- 



HYSTERIA. 



549 



cular spasms — are far less frequent. Of much interest are the 
involuntary movements which are now and again observed. I 
had a lady under treatment who, without wishing it, but with- 
out being able to resist the inclination, would for hours at a 
stretch keep on raising both arms and letting them fall again 
without the least feeling of fatigue. 

Clonic muscular spasms, in the muscles of the face as well 
as in the extremities, which, appearing in paroxysms, usually 
are symmetrical in their distribution, and are not sufficient 
to produce movements of the affected limbs, have been de- 
scribed by Friedreich as paramyoclonus multiplex, and by See- 
ligmiiller as myoclonia congenita. That they are of hysterical 
origin is more than probable. The trouble is rare, and is in 
most instances to be regarded as an emotional neurosis. The 
strength of the muscles and their electrical excitability remain 
unaltered, and sensory changes are absent. Sometimes there 
are tender points along the spine, which are best treated by 
the anode of the constant current. Other measures are not 
necessary, especially as recovery seems to be the usual out- 
come (cf. lit.). 

Closely related to though not identical with myoclonus is 
the group of symptoms which has of more recent years been 
described as " maladie des tics convulsifs." Irregular move- 
ments having the appearance of intended movements, but which 
have become automatic, occur in the face and in the extremi- 
ties ; they may be confined to one side. It is not improbable 
that imitation or even direct suggestion may play an important 
role in the production of these movements (Toharski, Neurol. 
Centralbl., 1893, 16). Mental abnormities are rarely absent in 
such patients ; thus we find " a tendency to the formation of 
fixed ideas — i. e., a low degree of mobility of the contents of 
consciousness and the frequent repetition of the same psychical 
processes " (Toharski). The movements present the following 
peculiarities : They have a psychical character ; they are re- 
peated in a monotonous manner ; they appear purposeful ; but 
since the will has nothing to do with their appearance, they 
occur without effort on the part of the patient ; at times they 
can be suppressed by an effort of the will. Fibrillary twitch- 
ings and involuntary contractions in certain muscles, especially 
in those of the face and the neck, more rarely of the hands, 
have been observed (Toharski). I have reported a case in 
which this condition was associated with allochiria, and which 



550 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

I succeeded in curing by verbal suggestion, at the International 
Congress in Rome (cf. Wiener med. Presse, 1894). 

LITERATURE. 

Starr, Allen. Paramyoclon. Mult., with a Report of a Case. Journ. of Nerv. and 

Ment. Diseases, 1887, xiv, p. 416. (The spasms appeared immediately after 

lifting heavy objects.) 
Rybalkin. Petersb. med. Wochenschr., 1887, 44, p. 366. 
Marina. Ueber Paramyoclon. mult, und idiopathische Muskelkrampfc Arch. 

f. Psych, und Nervenkh., 1888, xix, 3, p. 684. 
Ziehen. Ueber Myoclonus und Myoclonic Ibid., 1888, xix, 2, p. 465. 
Peiper. Ueber Myoclonic Deutsche med. Wochenschr., 1890, 19. 
Unverricht. Die Myoclonic Leipzig u. Wien, Deuticke, 1891. 
Goldflam. Neurol. Centralbl., 1892, 4. 

Weiss, M. Ueber Myoclonic Wiener Klinik., 1893, Heft 5. 
Jolly. Ueber die sogen. Maladie des Tics convulsifs. Charite-Annalen, 1892, 

p. 740. 
Buringh Boekhoudt et J. van der Weyde. Maladie des tics convulsifs. Weekbl. 

van het Nederl. Tijdschr. voor Geneesk., 1893, p. 369. 
Sciamanna. Malattia dei tic, R. Accad. med. di Roma, 1893. 

Often combined with paralyses of the extremities are joint 
contractures, which as a rule appear suddenly, and may per- 
sist for months and years. When occurring in the upper ex- 
tremities, in the elbow, in the wrist, and in the finger joints, 
they are usually flexor contractures ; in the knee and ankle 
joints, extensor contractures. The way in which they disap- 
pear under chloroform narcosis is very remarkable. Individ- 
ual muscles may also be the seat of contractures, and we have 
described a case on page 385 in which during the erect posture 
a contracture in the quadratus lumborum made its appearance, 
which disappeared when the patient lay down. 

LITERATURE. 

Zesas. Zur Differentialdiagnose der Gelenkneurosen. Chir. Centralbl., 1886, 
xiii, 16. 

Pitres. De l'analgesie chez les hysteriques a. l'etat de veille et dans le sommeil 
hypnotique. Journ. de med. de Bordeaux, 1886, 50. 

Miiller. Mitth. d. Vereines d. Aerzte in Steiermark, 1886, xxii. (Interesting 
Sensory Changes.) 

Lichtwitz. Les anesthesies hysteriques des muqueuses et des organes des sens 
et les zones hysterogenes des muqueuses. Paris, 1887. 

Falcone. Deutsche med. Wochenschr., 1886, xii, 41. (Spontaneous Falling 
Out of the Nails.) 

Ward. Philadelphia Med. and Surg. Rep., 1887, lvii, 5. (Hysterical Haemop- 
tysis.) 

Richer, Paul. Paralysies et contractures hyst., Paris, 1892, Doin. 



HYSTERIA, 



551 



Among the sensory disorders the diminution or complete 
loss of sensibility is the most important; this may be so exten- 
sive that the patients can feel nothing on any part of the sur- 
face of the body, not excluding the mucous membranes (con- 
junctivae, nose, tongue, mouth, vagina, rectum), so that one can 
touch them with the hot iron (thermo-cautery) or prick them 
with knives and needles, and they will not make the slightest 
sign or attempt to draw away the part; nay, more, there are 
cases in which the deeper tissues take part in the anaesthesia, 
so that folds of skin may be transfixed and fine needles thrust 
into the muscles down to the bone without the knowledge of 
the patient if she be blindfolded. Besides the general abolition 
of sensation, we may meet with circumscribed spots of anaes- 
thesia, anaesthetic zones, on the back, on the hands, etc. The 
hemianaesthesia, which is strictly confined to one side, and which 
implicates the mucous membranes as well as the skin, has already 
been mentioned. These sensory changes also may appear and 
disappear suddenly. 

Less common are the hyperaesthesias, which probably never 
take in the whole body, and never even one whole half of the 
body, but are usually confined to circumscribed areas, to cer- 
tain internal organs, or by preference to certain joints. These 
circumscribed areas, Charcot's hysterogenic zones, vary in 
their situation ; they may be on the back, on the chest, in the 
extremities, or elsewhere. Among the internal organs, in 
women the ovaries, in men and boys the testicles, are the parts 
that usually suffer. The ovarian hyperesthesia, which Charcot 
has studied very carefully, is closely related to the " major 
attacks " to be described later. That it is actually the ovaries 
which give rise to the acute pain when pressure is made over 
them Charcot has proved on pregnant women ; during preg- 
nancy the position of the ovaries is changed,. and it was found 
that there was a corresponding change in the position of the 
tender points. The women who suffer from this hyperaesthesia 
are in Paris called " ovariennes." 

Neuralgiform pains, which often affect the joints and which 
are very obstinate, are so common in the course of hysteria 
that whenever we find a joint neuralgia we should think of and 
search for a hysterical basis. Brodie has subjected them to a 
very accurate study, and has pointed out that it is at times ex- 
tremely hard to differentiate between a neuralgia and an actual 
disease of the joint. The hip and knee are most usually attacked. 



552 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

The joint is painful, especially on pressure or on motion ; hence 
such patients are, as a rule, found in bed or lying on the sofa. 
On closer examination the pain proves not to be confined to 
one spot, but to be distributed over more or less large areas of 
the lower extremity. The patient cries out if pressure is made 
in the neighborhood of the hip or the knee or lower down over 
the malleoli. She seems to be especially sensitive when watch- 
ing and following our manipulations ; but if the physician is 
able to divert her attention, pressure over an otherwise painful 
point will often evoke no complaint. In the course of the dis- 
ease the glutei may undergo some atrophic changes ; now and 
then transient swellings are noted. On the other hand, there 
are instances in which hysterical joints are the only cause which 
keeps the patient persistently in bed, and in which, in spite of 
an inactivity lasting for years, not a trace of atrophy can be 
recognized, while the general health shows no signs of impair- 
ment (cf. lit.). All these and other spontaneous pains, which we 
need not dwell upon here, occurring in hysterical individuals 
are to be regarded as being of psychical origin, and therefore 
as pain hallucinations (Strumpell, Hoist). 

Among the abnormities of the secretory organs, those 
which concern the urine chiefly deserve our attention. Hys- 
terical patients may urinate very little and not without diffi- 
culty (ischuria). On the other hand, we find some who urinate 
frequently and pass almost incredible amounts (cf. Mathieu, La 
polyurie hysterique, Revue neurol., 1893, 19). In the former 
case the specific gravity is high and the solid constituents of 
the urine are increased in amount. In the latter the urine re- 
sembles almost clear water. It would be erroneous to assume 
that the small or large amount of urine always depends upon 
the amount of water ingested. Indeed, patients who drink 
hardly anything may void very large quantities of urine, while 
those who drink a good deal may pass only a few drops at a 
time. Here, again, as with the manifestations of hysteria in 
general, no hard-and-fast rule can be given, nor can anything 
certain or constant be said about the salivary and sweat secre- 
tions, since they are equally subject to variations. 

Among the trophic disturbances we will only mention the 
hysterical oedema, which occurs as the white or as the blue 
type ; the former is soft in character, and the skin pits on pres- 
sure ; the latter is hard, is associated with diminished surface 



HYSTERIA. 



553 



temperature, and the skin sometimes presents a peculiar mot- 
tled appearance (Charcot ; cf. also Athanassio, Des troubles 
trophiques dans l'hysterie, Paris, 



The combined hysterical manifestations — that is, those 
originating- in the brain as well as in the spinal cord — consist of 
the so-called "paroxysms" or " attacks," in which conscious- 
ness is not lost, as in epilepsy, but which are associated with 
convulsions. Vague pains, ructus, yawning, the globus hys- 
tericus, ischuria, etc., may constitute the premonitory signs, 
which are immediately followed by violent respiratory move- 
ments, regular respiratory spasms, with laughing, screaming, 
weeping, barking, and finally the climax is reached in muscular 
spasms and convulsions resembling those of epilepsy. During 
such paroxysms the whole body may be thrown from side to 
side, and it may be impossible to restrain the patients, because 
they exhibit a strength far greater than that which they ordi- 
narily possess. After the fit — which may last from half an hour 
to an hour — has spent its force there follows a condition of gen- 
eral prostration, which usually does not last long and is fre- 
quently accompanied by polyuria. It is just these attacks 
which make the " home treatment " for hysterical patients so 
very difficult or finally even impossible. It has been found by 
experience that the sight of such patients — of the various 
contortions into which their bodies are thrown and the gro- 
tesque positions they assume — has an injurious effect on the 
other members of the family, especially if there are young 
girls among them. Such a scene and the consequent mental 
excitement have been known to cause similar attacks in other 
females. 

Duration and Course. — The duration and course of hyste- 
ria are by no means uniform, although this much may be said, 
that it is always chronic and may last for years and tens of 
years. There are patients who from the time of puberty until 
after the involution period are hysterical, and thus never attain 
to the full enjoyment of life. At the beginning of the trouble 
there is usually nothing more than a certain tendency to nerv- 
ousness, a certain proneness to eccentricities, annoying to the 
patient and still more so to the family. Soon various pains, 
which are apt to frequently change their seat and to vary in 
intensity, make their appearance, and certain respiratory phe- 



554 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

nomena, perhaps shortness of breath or a barking cough for 
which physical examination reveals no cause, begin to attract 
our attention. 

Severe motor disturbances are by no means noted in all 
cases; even contractures are not very common. On the other 
hand, there are few cases in which the sensibility does not at 
various times undergo striking changes. Anaesthesias and anal- 
gesias alternating with hyperassthesias and neuralgias, especial- 
ly of the joints, and persistent headaches, all help to sour the 
disposition of the patient. During menstruation the condition 
is usually aggravated. The patient is still more excitable than 
usual, and her complaints are louder. In those in whom the 
paroxysms are an important feature of the case this is more 
especially true, and sometimes the first menstrual period is the 
signal for the first attack, which is at regular intervals followed 
by others. In many cases of hysteria " attacks " never occur. 
The patients, indeed, may without any provocation have fits of 
crying, laughing, and screaming, but no convulsions. With 
advancing age, and when the sexual functions are becoming 
inactive, the hysterical phenomena fade. As the hair turns gray 
the disposition becomes calmer and more equable, and even 
egotistical, exacting, peevish women, who have tormented their 
families continually and who were extremely hard to manage, be- 
come yielding, amiable old ladies after the hysterical manifesta- 
tions have once left them. Still there are, unfortunately, excep- 
tions in which these persist even after the seventieth birthday. 

The prognosis may be inferred from our description of the 
course of the disease. Doubtful as it always is, it is made still 
more gloomy from the fact that persons who have for years 
suffered from hysteria are apt to be subject later in life to 
actual organic nervous diseases, especially of the brain (Feld- 
mann, Inaug.-Dissert, Leipzig, 1887). 

Hysteria was thought, as the name indicates, to occur ex- 
clusively in members of the female sex. That it is more preva- 
lent among them there can be no question, but Charcot and 
his pupils have shown convincingly that it does occur in men 
and boys, and that, too, much more frequently than might a 
priori have been supposed. From him we have learned that it 
occurs frequently among the French soldiers. Further inves- 
tigations may prove that this would hold good not only for 
the French but also for other armies. Age seems to have 
much less influence than was at first assigned to it. Hysteria 



HYSTERIA. 



555 



in children is by no means rare. The full development of all 
hysterical manifestations in the young, who are far from being 
sexually mature, proves that puberty and the sexual organs are 
of less importance in the causation of this neurosis than has 
formerly been supposed. 

LITERATURE. 

Debove. Gaz, des Hop., 1886, 20. (Hysteria in the Male.) 

Tuczek. Berliner klin. Wochenschr., 1886, xxiii, 31-33. 

Freud. Wiener med. Blatter, 1886, ix. (Hysteria in the Male.) 

Dreschfeld. Med. Chronicle, 1886, v, 3. (Hysteria in the Male after Trauma.) 

Duponchel. L'hysterie dans l'armee. Revue de med., 6 Juni, 1886, vi. 

Janssen. Nederl. Weekbl., 1887, ii, 13. (Hysteria in Soldiers.) 

Riesenfeld. Hysterie bei Kindern. Inaug.-Dissert., Kiel, 1887. 

Dubois. Schweizer Correspondenzbl., 1887, xvii, 13. (Hysteria in Men and 

Children.) 
Coustan. Arch, de med. et de pharm. mil, 1887, x, 5. (Hysteria in the Male.) 
Handford. British Med. Journ., October, 1887, 22. (Hysteria in a Male.) 
Moricourt. Gaz. des Hop., 1887, 6. (Hysteria in the Male.) 
Engelsberg. Wiener med. Wochenschr., 1888, xxxviii, 14. Hysteria in a Boy 

aged Thirteen.) 
Lees, David. Lancet, June 23, 1888, i. (Hysteria in Two Boys.) 
Ray. Hysteria in the Negro. New York Medical Record, July 2, 1888, xxxiv. 
Clark. Journ. of Ment. Sc, January, 1888, xxxiii. (Hysteria in the Male.) 
Bitot. L'hysterie male dans le service de M. Pitres a l'hopital St. Andre de 

Bordeaux, Paris, 1890. 
Reischauer. Inaug.-Dissert., Berlin, 1890. (Case of Hysteria in a Man after 

External Urethrotomy.) 
Sollier. La France med., 1891, 38. (Infantile Hysteria with Convulsive Form.) 
Duvoisin. Ueber infantile Hysterie. Inaug.-Dissert., Basel, 1891. 
Chaumier. Semaine med., 1891, 58. (Hysteria in the New-born (!) and in 

Children Two Years of Age.) 

Diagnosis. — The diagnosis of hysteria may at one time be 
very easy, at another we may encounter no inconsiderable diffi- 
culties. When we have an array of ever-varying symptoms 
occurring apparently without order, when the patients com- 
plain to-day of this, to-morrow of that, while the physical signs 
show no grounds for their troubles, it does not need an expert 
to suspect and diagnosticate a hysterical condition. If, on the 
other hand, the disease sets in suddenly without previous dis- 
orders of any kind, in one case with a hemiplegia, in another 
case with a severe hip trouble, it may be by no means easy to 
say whether and, if so, why the hemiplegia is of hysterical 
origin, and whether or not the joint affection is to be regarded 
as a hysterical coxalgia. 



556 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

The following points will in the majority of cases be found 
sufficient to clear up any difficulties which the diagnosis pre- 
sents : 

With regard to the cerebral symptoms, and more especially 
those belonging to the affections of certain of the cranial nerves, 
we have in previous chapters pointed out some features char- 
acteristic of the hysterical varieties. It will be necessary in 
every separate case to exclude scrupulously anatomical lesions 
and to determine whether there are in addition to those be- 
longing to the cranial nerves other symptoms which point to a 
hysterical condition. If such be found, and more especially if 
our objective examination gives negative results, the diagnosis 
of hysteria is warrantable. 

These rules are particularly applicable where we have to 
decide whether a hemiplegia is hysterical or due to a lesion in 
the internal capsule, whether a contracture has to be regarded 
as hysterical or cortical (page 184), and whether the disturb- 
ances of the respiratory organs depend upon diseases of the 
lungs or the larynx, or are to be referred to a neurosis of the 
vagus or of the recurrent laryngeal nerve. 

The recognition of the hysterical nature of spinal manifesta- 
tions belonging to the motor apparatus may give rise to the 
greatest difficulties. It is upon the electrical examination that 
we must rely in deciding whether the paralysis of an extremity 
depends or not upon a peripheral cause — that is, upon a neuri- 
tis. A well-marked reaction of degeneration always points to 
a chronic inflammatory condition. The age of the patient is 
of some value. Hysterical paralyses occur between the ages of 
fifteen and thirty, and more particularly in women. Further, 
we observe almost always associated with hysterical paralyses 
grave sensory disturbances which are not necessarily present 
in the other kinds (cf. Lumbroso, Lo Sperimentale, Firenze, 
1887; reference, Neurol. Centralbl., 1888, 7). The existence of 
muscular atrophy is not sufficient to determine the organic 
nature of the paralysis because an atrophy of muscles does not 
exclude hysteria, as we have pointed out above (Brissaud, 
Arch, de physiol. norm, et pathol., Avril, 1887, p. 339). Schlapo- 
bercki (Inaug.-Dissert., Berlin, 1893) has pointed out the sig- 
nificance of relapses in the hysterical paralyses. 

Contractures, if of hysterical origin, set in suddenly, and are 
almost always accompanied by other hysterical manifestations, 
meteorism, ovarian hyperassthesia, and ischuria. Where such 



HYSTERIA. c^y 

symptoms are absent we must be very careful in our exami- 
nation and take into account the possibility of an anatomical 
lesion either of central or of peripheral origin (cf. Blocq, Des 
Contractures, These de Paris, 1888; Progr. med., 1888, xx, p. 

397)- 

Hysterical muscular spasms may be taken for tetany, as 
the case of Caiger, in the Lancet of August 20, 1887, shows. 
To the frequent occurrence of rhythmical spasm in certain 
groups of muscles in hysteria, Pitres has drawn attention in an 
article in the Gaz. med. de Paris, 1888, 13. 

Trembling and shaking movements, which somewhat re- 
semble those of intention tremor as they become more marked 
on voluntary motion, have been noted, but are rare (Charcot, 
Progres med., 1890, 37). The possibility of mistaking such con- 
ditions for multiple sclerosis (or vice versa) should, however, 
always be kept in mind. In our account of the latter disease 
we shall come back again to the points for the differential diag- 
nosis between the two conditions. * 

The sensory changes in hysteria, the anaesthesias, affect, as 
we said, not only the skin, but also the deeper tissues, so that 
needles may be inserted down to the bone without being felt. 
Usually all qualities of sensation take part in the disorder, so 
that the so-called muscular sense is also lost and the patients 
are unable after closing their eyes to give any account of the 
position of their limbs. Pronounced anaesthesia is found dur- 
ing the hysterical paroxysms. An anaesthesia extending over 
the whole body and taking in all the mucous membranes is 
almost always hysterical in nature. These grave sensory dis- 
turbances render explicable the possibility that patients some- 
times for some reason or another produce sores on their own 
bodies. With regard to such lesions which may at times be 
mistaken for those of lupus or carcinoma the reader is referred 
to the Deutsche Med.-Ztg., 1892, 88 (Account of the session of 
the Berlin Medical Society, October 26, 1892). 

Hyperaesthesias and neuralgias occurring in hysteria are 
typical in that they are very changeable, so that to-day cranial, 
to-morrow spinal, nerves are the seat of the pain. Neuralgias 
of joints, if organic disease can be ruled out and if they are 
very obstinate and resist all the ordinary therapeutic measures, 
may be safely looked upon as hysterical. The " attacks " may 
be mistaken for epileptic fits. The important point to remem- 
ber in this connection is that in the hysterical attacks con- 



558 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

sciousness is never lost as completely as in epilepsy. Biting of 
the tongue is an exception in the former. The hysterical at- 
tacks are, moreover, attended with noisy laughing and cr} 7 ing, 
etc., while epileptics, with the exception of the initial cry 
(which is not constant), pass through the whole convulsive 
stage quietly and without uttering a sound. It has been 
claimed that there never occurs an elevation of temperature 
during the hysterical seizure, while the epileptic fit is accom- 
panied by a slight rise, 1.2 to i.8° F. This statement can not 
easily be controlled, and certainly needs further confirmation. 
Finally, it should be remembered that hysterical attacks may 
in some instances be produced by pressure upon the ovaries or 
the testicles, while in epilepsy this is never the case. 

Pathogenesis and ^Etiology. — About the nature of hysteria 
we are absolutely in the dark. Not one of the many attempts 
to explain the disease can be regarded as more than a vague 
hypothesis. This one fact may be regarded as certain, that 
the existence of grave anatomical changes is excluded, or, at 
any rate, is highly improbable, otherwise the suddenness with 
which the symptoms come and go would be absolutely inex- 
plicable. The old idea that the uterus must be held responsi- 
ble in every case and under all circumstances for the disease, 
which was consequently called hysteria (vo-repla), has been 
shown to be untenable by the number of cases observed in 
men and young children ; and the more cases we see, the clearer 
it becomes that the hysterias occurring in males and in little 
children furnish a considerable proportion of the total number, 
and the more ridiculous becomes the term " hysteria," which 
sooner or later will be given up completely. The influence of 
the sexual organs on the disease will be discussed later, but we 
would state emphatically that the opinion that these are always 
the starting point of the disease is indefensible. 

But how shall we explain the disease? If we agree that all 
symptoms of hysteria have certain characteristics in common, 
they may perhaps all together be traced to an increased excita- 
bility of the whole nervous system, to the quicker response 
to stimuli from without and within. Just as we have morbid 
conditions in which the excitability of the nerves and the mus- 
cles to the electrical current is found to be increased, we may 
imagine also an analogous condition in w^hich all the nerves, 
including the nerve elements of the central organs of the brain, 



HYSTERIA. 



559 



especially of its cortex, those of the spinal cord, and also of the 
peripheral nerves, are in a constant state of abnormal or patho- 
logical excitability. That in such a state the imagination plays 
an important role is self-evident — not, however, in the sense that 
all the sufferings of which the patient complains are imaginary 
and merely depend upon the imagination ; we rather mean that, in 
the condition described, the ideas are consciously or unconscious- 
ly influenced by the will, they are formed and disappear more 
quickly and are constantly changing. Such a quick and unnat- 
ural change can not but exert an unfavorable influence, first upon 
the mind and disposition, and later upon the bodily condition. 

In reality it is in the majority of cases a disturbance of the 
psychical equilibrium which produces the disease. It is not 
impossible that careful study of the astiology may do much 
toward a clearer understanding of the nature of the malady ; 
Guinon has shown this in his excellent monograph, Les agents 
provocateurs de l'hysterie, Paris, 1889. The causes may be 
subdivided into direct and indirect. To the former belong a 
hereditary, physical as well as psychical, predisposition of the 
individual. There is no doubt that only those persons can 
become hysterical who are from birth so predisposed, because 
they have a nervous system which presents the peculiarities 
that we have just described. This congenital, because heredi- 
tary, predisposition finds favorable conditions for further devel- 
opment in (a) sex, (J?) age, (c) education, (d) nationality or race 
of the patient. That the female sex and those just arriving at 
the age of puberty are prone to the disease we have said before, 
although the male sex and other periods of life besides that of 
puberty are by no means exempt. The hysteria which occurs 
in early childhood, and which has been observed between five 
and ten years of age, deserves special study. 

Much must be attributed in the causation of hysteria to a 
faulty education. The brain may be overtasked at the ex- 
pense of the body, and, in consequence of too little firmness 
on the part of the parents, capriciousness, inconsiderateness, 
lack of truthfulness, of energy, and of will power are fostered 
in the child, and, finally, when the children have behaved badly, 
the mysterious threats, especially of injudicious servants, of 
sending after them wild beasts, ghosts, "the black man," etc., 
can drive them into such a chronic state of fear that they can 
not go into a dark room without palpitation and the most in- 
tense feeling of terror. All such and many other mistakes in 



560 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

the early education of the child become indirectly causes of 
hysteria. The occupation may have an influence if it be asso- 
ciated with bodily and mental overexertion, and in certain call- 
ings the possibility of intoxication (lead, mercury, bisulphide of 
carbon, etc., must not be forgotten) (Rouby, Contribution a 
l'etude de l'hysterie toxique, These de Paris, 1889). As to 
race the Slavonic (Poles, Russians), the Latin races (the French 
and the Italians), and, above all, the Semitic peoples, are more 
liable to hysteria than the Teutonic. The severest forms of 
hysteria are seen in French women and in Polish Jewesses. 
This may depend upon the national characteristics ; the lively, 
impetuous temperament which we find on an average more 
frequently in the Slavs, etc., than in the Teutons, forms a par- 
ticularly favorable soil for the development of hysteria. 

Among the direct causes disorders of the sexual organs 
play the most important part, and in both sexes this factor is 
equally potent. We must not think that the affection, which, 
especially in women, may, from a gynaecological standpoint, be 
very insignificant — for instance, a flexion, or a change in posi- 
tion of the uterus — has in itself much to do with the matter ; it 
is much rather the idea that the trouble exists, and the anxiety 
lest it should interfere more or less materially with coitus and 
parturition, which constitute the direct cause of the depres- 
sion of spirits. The conjugal obligations — coitus, pregnancy, 
parturition — play such an important role in the life of every 
woman, if she has not missed her calling, that the mere idea 
that the sexual organs are diseased or incapable of performing 
their function is sufficient to give a severe shock to her hap- 
piness. In a man it is much less the potentia generandi than 
the potentia coeundi that causes him anxiety. The above-men- 
tioned psychical impotence, if it exist for a long time, in itself 
suffices to bring about a hysterical condition, and sexual neu- 
rasthenia is not rarely accompanied by pronounced hysterical 
manifestations, so that we can well speak of a coexistence of 
the two diseases. 

Secondly, fright ought to be mentioned as a direct cause 
of hysteria ; a girl upon whom an attempt at rape has been 
made, or a man who has been attacked by a robber, may be- 
come the subject of a hysteria, which may last for years, or 
may even be incurable. It is not necessary in such cases that 
fright be associated with any trauma, the mental shock suffi- 
cing to produce all the symptoms. 



HYSTERIA. 



56l 



If bodily injuries are associated with fright the parts affected 
frequently become the seat of hysterical disorders. Thus, with 
a history of a lesion of the hip joint, after the injury has long- 
been recovered from, we may find a hysterical coxalgia, etc. 

It is important to recognize the fact that an injury inflicted 
upon a person who is already suffering from hysteria or who 
by heredity is predisposed to the disease, may be followed 
by different consequences than would be the case in a normal 
individual. Thus a fall on the back which has produced noth- 
ing more than a contusion of the soft parts may, in a hysterical 
individual, lead to a monoplegia or a paraplegia of the lower 
extremities, while such an accident would have had no such 
results in a healthy individual. I have seen a number of such 
cases, to which the term hystero-traumatic affection, rather 
than traumatic hysteria, would be applicable. In this connec- 
tion the paper of Miura, Sur trois cas de monoplegie brach. 
(Arch, de Neurol., 1893, xxv, 75), should be mentioned. 

The psychical traumatism may be of such a nature as to 
have an immediate influence, or may act gradually and insidi- 
ously. Among the former we have fright, emotions of anger, 
rarely of joy ; to the latter belong grief, anxiety, wounded self- 
respect or vanity, and the like. 

A special kind of neurosis due to fright has of recent years 
been much discussed and carefully studied by many investiga- 
tors, although thus far no unanimous conclusions in regard to 
its nature have been arrived at. To this condition which thirty 
years ago was described under the name of commotio medullas 
spinalis, or railway spine, the term " traumatic neurosis " is now 
often applied. Certain investigators claim that the affection is 
an entity per se which, like any other distinct disease, should 
have its own name ; oihers disagree on this point and regard 
the old name as sufficient ; still others consider both terms to 
be incorrect, and simply speak of an " accident neurosis." 

Whether this affe-ction is to be regarded as a form of hys- 
teria is a question of very little practical importance. It is 
certain, however, that aetiologically as well as symptomatically 
the two conditions show much that is alike. The " traumatic 
neurosis " is produced by the fright alone, the bodily trauma 
is a non-essential ; the latter may be present or not, but the 
neurosis appears if the psychical shock has been sufficient. 
Hence we see that serologically we have here the same factor 
that frequently leads to hysteria. So far as the subsequent 
36 



$62 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

manifestations are concerned the results are similar ; the sub- 
jective symptoms more especially are often of a typically hys- 
terical character, though neurasthenic disturbances are also 
encountered. Motor and sensory disorders are met with. 
Among the former may be mentioned a general motor weak- 
ness, an abnormal proneness to fatigue, among the latter pain 
in the head and back, paraesthesias, hyperesthesias, and anaes- 
thesias. Narrowing of the visual field, diminution in acute- 
ness of vision, photophobia, disorders in color vision or hyper- 
esthesias of the auditory, olfactory, and gustatory nerves have 
been observed. Again, we may find cutaneous anaesthesias, 
situated chiefly on the back, in the shape of irregular plaques, 
or having the distribution of a well-marked hemianaesthesia 
hysterica ; at other times, again, they may extend over the 
head, neck and upper chest (doll's head form). In all cases, 
however, the results of two separate examinations may differ 
as the anaesthesia may shift its place or vary in extent. The 
rules for making sensory examinations have been excellently 
formulated by Goldscheider (Neurol. Centralbl., 1892, 12): 
The skin and the tendon reflexes vary as they do in hys- 
teria. Urinary symptoms may be present or absent. While 
walking, and in general in making any motion, the patient 
avoids all movement of his spinal column. He fixes his 
trunk and moves with his back held stiff, using his hands as 
much as possible whenever he wishes to change his position 
(Oppenheim). 

Psychical abnormities appear chiefly under the form of de- 
pression, fear, irritability, hypochondriacal depression, and the 
like ; these symptoms are, however, not always due to the ac- 
cident, but often result from the trouble and annoyance entailed 
by the interminable negotiations before the degree of disability 
and the amount of damages to be paid are settled upon. 

It must be remembered that every patient with a so-called 
traumatic neurosis who has any damages to claim is suspected, 
if not of simulating, at least of exaggerating his symptoms, and 
it is certainly well for the physician to be cautious. On the 
other hand, it would be absolutely wrong to regard all such a 
patient's complaints simply as exaggerations 01 lies. We must 
examine him carefully, and in no case should an expert opinion 
be given after a single examination (Burchardt, M. Prakt. 
Diagnostik der Simulation von Gefiihlslahmung, Schwerhorig- 
keit und Schwachsichtigkeit, Berlin, Enslin, 1891). 



HYSTERIA. 



563 



The objective symptoms which are frequently, though not 
regularly observed, are, of course, valuable for the purpose of 
excluding simulation. They are : (1) The concentric narrow- 
ing of the visual field (especially for red and green) when this 
is found to be constant on repeated examinations (Schmidt- 
Rimpler, Deutsche med. Wochenschr., 1892, 24); (2) a peculiar 
narrowing of the visual field which was first described by 
Forster in cases of anaesthesia retinae. The value of this 
symptom has recently been pointed out again by Konig (Ber- 
liner klin Wochenschr., 1 891, 31) and by Placzek (ibid., 1892, 35). 
" The essential features of this symptom may be thus summa- 
rized : Objects moved into the field from the periphery to the 
centre can be seen farther out than those which are moved in 
the opposite direction ; if the patient fixes the white spot of 
the perimeter and we now make two examinations, in the one 
bringing the object in from the periphery and marking the 
points at which it becomes visible, in the other moving the 
object from the centre to the periphery and marking the points 
at which the object ceases to be seen, we shall obtain two fields 
of vision of unequal size, the former being the larger in every 
direction " (Konig). Simulation is here excluded unless the 
patient knows the symptom and has practiced with the perim- 
eter. (3) We find that if we press on painful points (in traumatic 
neuralgia) the heart's action becomes increased so that the 
pulse may rise from nineteen to thirty beats to the quarter of a 
minute (Mankopff), a condition which can only very rarely be pro- 
duced at will by the patient. The absence of Mankopff 's symp- 
tom does not, however, necessarily prove simulation (Strauss, 
Berliner klin. Wochenschr., 1892, 48). (4) Rumpf has described 
a sign which he has called " traumatic reaction of the muscles." 
If a strong faradic current be allowed to pass through a (pain- 
ful) muscle for from one to two minutes, the muscle does not 
at once return to its position of rest, as it would under normal 
conditions, but presents for a considerable time fibrillary or 
even clonic twitchings (Deutsche med. Wochenschr., 1890, 9). 
If we add (5) the quantitative diminution of the galvanic exci- 
tability of the motor nerves which has also been pointed out 
by Rumpf (Joe. eit\ we have at our command means sufficient 
to meet the attempts of simulators, who, according to some 
physicians, are constantly increasing in number. 

Among all these symptoms there is, with the exception of 
the traumatic reaction of the muscles, not one that is pathog- 



564 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

nomonic, and the clinical picture, which we possess, is not 
sufficiently definite to warrant us in regarding the affection as 
a disease by itself. After a personal experience with sixty- 
eight cases, and after a perusal of the literature, I must still 
regard it as belonging to the category of hysteria, an opinion 
which is not shaken by the fact that Schmaus has described as 
following spinal concussion anatomical changes consisting in 
a necrosis of the axis cylinders, which often occurred long 
after the trauma (Schmaus, Miinchener med. Wochenschr., 1890, 
28 ; also Arch. f. klin. Chir., 1891, xlii, Heft 1). In all cases of 
hysteria, particularly in the neurosis produced by fright, we 
can scarcely be cautious enough in our prognosis. It is always 
very uncertain so far as complete recovery is concerned, 
especially in individuals who are badly endowed psychically, 
in cases with a bad heredity, and in alcoholics. It may also be 
said that the harder the former occupation of the patient the 
worse, cceteris paribus, is the prognosis. 

With regard to the very important and difficult practical 
questions we may with Romer (Irrenfreund, 1889, xxi, 9, 10) 
mention the following : 1. Is the disease the consequence or 
the exclusive consequence of the accident? 2. Is it curable, 
and, if so, in what time? 3. Will the patient be completely or 
partially incapacitated ? The discussion of such questions can 
not here be entered upon ; the general points of view from 
which they can be answered will be found, however, in what 
has been said above. 

LITERATURE. 

Charcot. Progr. med., 1885, xiii, 18. 

Oppenheim. Arch. f. Psych, u. Nervenkh., 1885, xvi, 3. 

Troisier. Gaz. hebdom., 1886, 2. ser., xxiii, 18. (Hysterical Paralysis as a Re- 
sult of Trauma.) 

Charcot. Wiener med. Wochenschr., 1886, xxxvi, 20, 21. (Hysterical Coxalgia 
as a Result of Trauma in a Man.) 

Debove et Catrin. Remarques sur l'hysterie traumatique. Gaz. hebdom., 1887, 
2. ser., xxiv, 43. 

Vibert. Ann. d'Hyg. publ., Dec, 1887, xviii, 12. (Railway-Spine considered 
from a Medico-legal Point of View.) 

Lyon. Encephale, 1888, viii, i. (Hysteria after Grave Trauma.) 

Charcot. Arthralgie hystero traumatique du genou. Progres med., 1888, 
xvi, 4. j 

Bernhardt, Deutsche med. Wochenschr., 1888, 13. 

Strumpell. Ueber die traumatischen Neurosen. Berliner Klinik, Fischer, 1888, 
xvi, Heft 3. 

Grasset. Hystero-traumatisme. Lecons recueillies, Montpellier, 1888. 



HYSTERIA. 



565 



Opfer, F. Beitrag zur Lehre von den traumat. Affectionen des Riickenmarks. 

Inaug.-Dissert., Berlin, Schade, 1888. 
Bnginsky. Berliner klin. Wochenschr., 1888, 3. 
Wolff. Ueber Railway-Spine. Deutsche Med.-Ztg., 1888, 79, 80. 
Bernhardt. Von den allgem. u. traumat. Neurosen. Berliner klin. Wochenschr., 

1889, 13. 
Stepp. Deutsche med. Wochenschr., 1889, 4. 

Grasset. Lemons sur l'hystero-traumatisme. Paris, Lecrosnier, 1889. 
Meyer, Moritz. Berliner klin. W T ochenschr., 1889, 5. 
Auerbach. Die traumatische Hysterie beim Manne. Inaug.-Dissert., Strass- 

burg, 1889. 
Striimpell. Ueber traumatische Hysterie. Munch, med. Wochenschr., 1889, ii. 
Eisenlohr. Berliner klin. Wochenschr., 1889, 52. 
Guth. Ueber den diagnostischen Werth einzelner Symptome der traumatischen 

Neurose. Inaug.-Dissert, Berlin, 1890. 
Cramer (Eberswalde). Mimch. med. Wochenschr., 1891, i, 2. (Contains an 

excellent summary of the more recent papers.) 
Schultze, Fr. Ueber Neurosen und Neuropsychosen nach Trauma. Volkmann's 

Samml. klin. Vortr., 1891, iv, v, 14. 
Page. Railway Injuries. London, Giffon & Co., 1891. 
Wichmann, Ralf. Der Werth der Symptome der sogen. traumatischen Neurose 

u. s. w. Braunschweig, Vieweg, 1892. 
Oppenheim. Die traumat. Neurosen u. s. w., 2. Aufl. Berlin, Hirschwald, 

1892. 
Freund, C. S. Ein Ueberblick uber den gegenwartigen Stand der Frage von 

den sogen. traumat. Neurosen. Volkmann's Samml. klin. Vortr., n. F., 

1892, 51. 
Friedmann. Munch, med. Wochenschr., 1893, 20. 
Bruns. Neuere Arbeiten uber die traumat. Neurosen. Schmidt's Jahrb., 1892, 

ccxxxiv, p. 25 ; 1893, ccxxxviii, p. 73. (This paper is indispensable for any 

one making a special study of the subject.) 

The symptoms that appear after a person has been struck 
by lightning- sometimes resemble the array of symptoms ob- 
served in traumatic neuroses. Paralyses in the nerves of spe- 
cial sense, and motor and sensory paralyses, appear and last for 
a shorter or longer time. In the spring of 1889, when thun- 
derstorms were so frequent, I had the opportunity of examin- 
ing a man who, as a consequence of being struck by lightning, 
on recovering consciousness after three quarters of an hour, 
presented loss of the power of sight and smell on the side on 
which the lightning had entered and left the body, while on 
the same side hearing was diminished, and there was total an- 
aesthesia. These symptoms were associated with an obstinate 
insomnia. By hypnotism, frequently repeated, we were enabled 
to lessen this insomnia, and under the use of the galvanic cur- 
rent and the faradic brush the hemianassthesia disappeared. 



$66 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

The nerves of special sense implicated became fully normal 
after a month's treatment. In this case motor disturbances 
were never seen. According to the investigations of Limbeck 
(Prager med. Wochenschr., 1891, 13), we have to distinguish 
between direct and direct paralyses due to lightning ; he re- 
gards only the former as due to an action upon the nervous 
system, and has observed that the sensory paralysis disappears 
sooner than the motor. For further symptoms in such cases 
and for the post-mortem conditions found after death by light- 
ning, we would refer the reader to Schmitz's article in the 
Deutsche Med.-Ztg., 1887, 73, 74, in which further references 
on the subject may be found. 

Treatment. — The treatment of hysteria is always a very 
tedious matter, and for the physician sometimes the most tire- 
some and thankless task imaginable, and one to which he should 
only devote himself if he be assured of the implicit confidence 
of his patient, so far as this is possible in the case of hysterical 
individuals. This confidence is indispensable because the treat- 
ment of the disease does not consist in the main in the admin- 
istration of drugs in a routine fashion — valerian, asafcetida, cas- 
toreum, and the nervines — but must depend more upon the 
psychical influence by which we endeavor to diminish the ab- 
normal sensitiveness of the patient to external and internal 
stimuli, to arouse her energy, and to strengthen her will pow- 
er. This is, we admit, much more easily said than done, and 
we shall often have to confess that the patient's views about 
her trouble have not changed in the least, that she is as irri- 
table as ever, that her moodiness and capriciousness are in no 
way improved in spite of all our lectures — in a word, that we 
have obtained no positive result after " preaching reason " for 
hours. Still, we must not allow ourselves to become discour- 
aged, but ever again and again renew our efforts to obtain the 
desired end. 

If we clearly see that these are fruitless, and especially if 
we are convinced, as is often the case, that the family, far from 
assisting the physician, are virtually acting against him during 
his absence, we must impress upon them the necessity of re- 
moving the patient to some institution. French physicians lay 
the greatest stress upon isolation in such cases, and are inclined 
to attribute the relatively favorable results of their treatment 
to this factor. In this country people are not so easily per- 



HYSTERIA. 



567 



suaded to agree to this procedure as in Paris, where in the city 
itself or in the suburbs there are various admirably conducted 
institutions which receive only hysterical patients. With us, 
therefore, home treatment ought first to be tried. In France 
this is usually discarded from the first. It is a different matter, 
of course, if we have to deal not with a mild degree of hys- 
teria, but with hystero epilepsy and major attacks. Then a 
transference to an institution, as soon as practicable, ought to 
be urged. 

The bodily treatment may be either general (that is, direct- 
ed to the nutrition, to the condition of the blood, and the 
strength of the patient) or symptomatic (that is, intended to re- 
lieve the troubles of the patient as they arise). In the treat- 
ment of contractures we should never make use of plaster-of- 
Paris bandages (Charcot). 

With reference to the nutrition, it was Weir Mitchell and 
Play fair who first recommended absolute rest in bed, with 
massage, electricity, and copious feeding. Their patients were 
forced to take considerable quantities of milk, meat, bread, etc., 
and it was found that with the increase of the body weight the 
hysterical symptoms and attacks diminished. Of late years 
good results have been obtained from this practice by Bins- 
wanger (Allgem. Zeitschr. f. Psych., 1883, xl, 4), and the com- 
munications of Leyden (Berl. klin. Wochenschr., 1886, xxiii, 16) 
and Burkart (ibid., 1886, 16) should encourage us to further 
trials with this method, although as far as my own experience 
goes the results have by no means always been brilliant. The 
cases in which the excessive ingestion of food was badly borne 
and led to a disagreeable gastric catarrh were by no means un- 
common, and even where the food was well assimilated the de- 
sired results were not always obtained (cf. also Gilles de la Tou- 
rette et Chatelineau, La nutrition dans l'hysterie, Progres med., 
1 888, viii, 48 ; 1 889, ix, 18, 19, 3 1). That much attention has to be 
paid to the nutrition there can be no question, and the increase 
in the body weight usually can be regarded as a favorable indi- 
cation. To attain this, however, in many cases, not absolute 
rest, but, on the contrary, systematic muscular exercise is need- 
ed. Well-regulated home gymnastics, undertaken according 
to definite principles (Schreber, Angerstein, and Eckler), are to 
be preferred and will be often found an excellent means of 
combating the distressing insomnia. 

In certain cases, to be selected of course, with care, general 



568 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

faradization as recommended by Beard and Rockwell is of 
great service. The patient for this purpose is placed upon a 
stool with his bare feet upon a moist large electrode, which is 
connected with the negative pole of the secondary coil. With 
the anode, which consists of a large sponge electrode, all parts 
of the body are treated in succession. Instead of the moist we 
may avail ourselves of a dry electrode in the form of a soft 
brush. The pain which is caused by the latter method is, at 
least with strong currents, quite considerable ; nevertheless, the 
method deserves warm recommendation in certain hysterical 
affections and especially in joint neuralgias. 

About the influence and the value of static electricity as a 
therapeutic agent our experience is not sufficient to warrant 
any definite conclusions. It is not easy to judge of the useful- 
ness of the treatment, as it is usually combined with other 
measures, the therapeutic significance of which must not be 
left out of consideration. Whether the action of static elec- 
tricity differs essentially from that of the faradic and galvanic 
current, and, if so, in what this difference consists and under 
what circumstances the one or the other is indicated, we are 
not as yet in a position to say. Clemens has used it with good 
results in cases of hysterical aphonia by applying one pole with 
condensers directly over the muscular branches of the accesso- 
rius as spark-producing electrode (Therap. Monatshefte, 1890, 
iv, Heft 8, p. 402). 

It is rare that we treat a case of grave hysteria without at 
one time or another during the course of the disease being 
obliged to resort to massage — for one thing, because the pa- 
tient desires as much variety as possible ; but at the same time 
we must not overlook the fact that by its use many of the pa- 
tient's troubles are considerably relieved. This is not the place 
to enter into the minute details of this method of treatment. 
They may be found in the writings of Schreber, Reibmayr, 
Zabludowski, and others. 

The cold-water treatment is indicated where we desire to 
harden the constitution against external influences, changes of 
temperature, etc. We should be very careful, however, in em- 
ploying low temperatures, and the water with which the pa- 
tient is sponged or in which hip baths and the like are taken 
ought to be at least 8o° F. For the use of ice-cold douches, in 
the way recommended by the French, certain facilities are 
requisite. The pressure of the water should be very great and 



HYSTERIA. 



569 



the duration of the bath should be so short (from ten to fifteen 
seconds) that the patient has not time to become aware how 
cold the water really is. I have watched this practice repeat- 
edly in some of the well-known hydrotherapeutic establishments 
of Paris, and have had occasion to notice the immediate benefi- 
cial effects following the application. The lasting results, as 
Charcot and others are quite convinced, are so marked that (in 
Paris) cold douches are considered to be indispensable in the 
treatment of hysteria. It would be a very desirable thing if 
the necessary arrangements for this treatment could be intro- 
duced into our hydrotherapeutic institutions. The ordinary 
shower bath, which comes down upon the patient just about 
like rain, is, of course, not sufficient. In the treatment of some 
of the particularly distressing symptoms it is, of course, in the 
first place the paroxysms which deserve our attention, because 
they, more than any other of the hysterical phenomena, are 
liable to render home treatment almost impossible. We may 
sometimes be able to cut short an attack by steady pressure 
with the hand over the ovaries continued for some time, but 
this can be better accomplished by allowing the patient to in- 
hale a little chloroform. To guard against a repetition of the 
attacks we have no reliable means, yet cool prolonged baths 
with affusions of colder water deserve a thorough trial. If 
these do not seem to be beneficial, and if the patient complains, 
before the onset of every attack, of pains in the ovarian region, 
and if we, moreover, can succeed in bringing about an attack 
by pressure over the (tender) ovaries, the question of oophorec- 
tomy has to be considered. The family relations, especially 
the sterility which naturally follows the operation, have to be 
taken into consideration, nor should we forget that the opera- 
tion has often by no means been followed by the desired effect, 
although the fact that it frequently exerts a favorable influence, 
as Hegar and Schroder have seen, can not be questioned. 
Whether the ovaries are actually diseased or not is altogether 
of minor importance. It is the presence of pain immediately 
before or after the attack in the region of these organs which 
should suggest an operative interference. Cauterization of the 
clitoris, advised by Friedreich, is a procedure which should 
only be resorted to in the most exceptional cases. In all in- 
stances the sexual organs ought to be carefully examined, and 
small operations, such as dilatation of the cervical canal, repo- 
sition of the uterus when in a position of flexion or version, if 



570 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



indicated, should be undertaken. Vaginismus, if it exists, should 
also be treated. 

The motor and sensory disturbances have to be met in 
the manner indicated above. In cases where we suspect ma- 
lingering or willful exaggeration, procedures which are disa- 
greeable or even painful are to be preferred — for instance, the 
cold baths, the faradic brush, the actual cautery, etc. The 
more minute details of the treatment must be left to the per- 
sonal tact of the physician, whose capability of individualiza- 
tion, of treating every case by and for itself, should make it 
unnecessary for us to enlarge upon all the principal phases of 
this disease. With regard to the internal medication, let it 
suffice to warn against the use of narcotics, especially morphine, 
which can not be given in a disease of such long duration in 
effectual doses without creating the habit. 

LITERATURE. 

Stein. Die allgemeine Elektrisation des menschlichen Korpers. Halle, 1883, 

2. Aufl. 
Widmer. Schweizer Correspondenzbl., 1886, xvi, 9-1 1. (Cure through Castra- 
tion.) 
Reibmayr. Die Technik der Massage. Wien, 1886, 2. Aufl. 
Tait, Lawson. Lancet, 1887, ii, 25. (Cure effected by the Removal of 

Pessaries.) 
Pitres. Progres med., 1887, xv, 8. (Static Electricity.) 
Greffiee. De l'electricite statique et de ses applications a la therapeutique. 

Paris, 1887. 
Zabludowski. Zur Indication und Technik der Massage. Berliner klin. Wochen- 

schr., 1887, 36. 
Burkart. Berliner klin. Wochenschr., 1888, xxiv, 45-47. 
Gittermann. Deutsche Med.-Ztg., 1888, ix, 24. (Feeding System.) 
Didier. Sur l'electricite faradique dans l'Hysterique. Lyon med., 1888, lviii, p. 

356. 
Dutton. Lancet, June 13, 1888, i. (Massage, Feeding System, Seclusion.) 
Bielschowsky. Ueber Influenzelektricitat, etc. Therap. Monatsh., Marz, 1889. 
Hirt. Lehrbuch der Elektrodiagnostik und Elektrotherapie. Stuttgart, Enke, 

1893, pp. 187 et seq. 



CHAPTER III. 

EPILEPSY — FALLING SICKNESS — MORBUS SACER — MORBUS COMITIALIS. 

The term epilepsy is often misused, inasmuch as it is ap- 
plied not only to the genuine classical epilepsy, but also to many 
conditions, characterized by convulsive attacks, in which on 
careful examination we can detect various other abnormities, 
and which, unlike genuine epilepsy, have a tangible cause. If 
a person in consequence of traumatism, of fright, of peripheral 
irritation (pressure upon a sensitive scar), or in consequence of 
cerebral syphilis, etc., becomes " epileptic " — that is to say, suf- 
fers from convulsions with or without loss of consciousness — 
these convulsions clinically may resemble very closely those of 
genuine epilepsy, but pathologically as well as genetically the 
two conditions are entirely different. 

For all such cases the term "epilepsy" is unjustifiable. 
Traumatic epilepsy, fright epilepsy, and reflex epilepsy are 
not genuine epilepsy. The difference is still greater between 
the so called Jacksonian and the genuine epilepsy. Injackso- 
nian epilepsy the convulsive attacks depend upon a disease of a 
portion of the cortex. Hence the term "cortical epilepsy" is 
also applied to this condition (cf. p. 186). 

The genuine epilepsy is a general neurosis, and we do not 
know that it ever produces a permanent anatomical alteration 
in the brain, and that the changes are not rather molecular in 
character, appearing from time to time in the brain, most prob- 
ably in the brain cortex, and leading to the "epileptic attack" 
and then disappearing again. About the role of auto-intoxica- 
tion we shall speak later. 

Etiology. — We are not acquainted with any essential cause 
for classical epilepsy. Physicians with a large experience have 
often enough occasion to see genuine epilepsy develop without 
there being any appreciable etiological factor. 

57i 



572 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



It has been the custom of most writers to distinguish predis- 
posing or general from exciting or special causes ; only the 
former are of importance. The latter have an influence only 
upon the frequency and the severity of the individual attacks, 
but are never responsible for the production of the disease. 
Among the former heredity has been given the first place, and 
there is no doubt that hereditary neuropathic tendencies in- 
crease the susceptibility to nervous diseases in general and cer- 
tainly to epilepsy ; but this heredity does not by itself suffice 
to make of an otherwise healthy individual an epileptic. For 
this usually an additional cause is needed — for instance, syphi- 
lis. If an individual with hereditary tendencies acquires syphi- 
lis, he is more likely to become epileptic — that is, to suffer from 
a genuine epilepsy, which is neither preceded nor followed by 
any appreciable anatomical changes, either in the brain or in 
its vessels — than a person infected with the same disease but 
burdened with no family taint (cf. Kowalewsky, Berliner klin. 
Wochenschr., 1894, 4). Important, therefore, as heredity may 
be, it is in itself not sufficient to constitute a cause for epilepsy. 
The manner in which the tendencies were acquired is also 
irrelevant, and the question whether the father or mother, or 
both were given to alcoholism, and whether both or either of 
the two was intoxicated at the moment of generation of the 
child has no significance. Notwithstanding the relative fre- 
quency with which epilepsy occurs, the number of cases would 
be much larger if either of these factors could have a decided 
influence in the causation of the disease. 

Age and sex seem to be of little moment in this connection. 
Although it is true that in the majority of cases the disease 
affects individuals in the first half of their lives, more especially 
between the ages of ten and twenty, the attacks may begin 
much later and may not appear until after the age of forty or 
fifty. Indeed, cases in which the first convulsion made its ap- 
pearance between the sixtieth and the seventieth year have been 
recorded (cf. Mendel, Die Epilepsia " tarda," Deutsche med. 
Wochenschr., 1893, 45). With regard to sex, it has been noted 
that during the period of puberty, between twelve and sixteen, 
more girls than boys become epileptic ; if, however, the aver- 
age of all cases be taken, the difference between the numbers in 
the two sexes is very slight, and in early childhood — from the 
fourth to the seventh year — it is nil, the cases being equally 
distributed between the two sexes. 



EPILEPSY. 



573 



Among the so-called exciting causes intercurrent gastric 
affections play a very important part : overloading of the stom- 
ach or the ingestion of unusually indigestible food often pro- 
duces an "attack" which without this setiological factor would 
have occurred, only later, or perhaps not at all. I have had for 
years a gentleman under observation who after eating pork 
and beans invariably has an attack a few hours later. Indi- 
gestion is all the more hurtful if the stomach has been over- 
loaded before going to bed. 

Certain substances which are taken into the system, whether 
as food or for the sake of their agreeable effects, or again as 
medicines, are very dangerous to the epileptic. Among these 
are alcohol, mushrooms, certain spices (cayenne pepper and 
paprika), also all narcotics, more especially, as we have learned 
in more recent years, cocaine. The " cocaine epilepsy " has been 
described by Heimann (Deutsche med. Wochenschr., 1889, 12). 
Under certain circumstances other medicines — antipyrine, for 
example — may act as poisons and provoke an epileptic attack 
(cf. Tuczek, Die Antipyrin-epilepsie, Berliner klin. Wochenschr., 
1889, 17). In view of the wide employment of antipyrine with- 
in a comparatively short time since its discovery, and the popu- 
larity which it enjoys, on account of which it is used in all pos- 
sible kinds of perfectly different diseases, this observation must 
be regarded as possessing great practical importance. 

It is generally known that anything which exerts a sud- 
den influence upon the cerebral circulation may be the direct 
cause for an individual attack, although it is an open question 
whether the blood current is accelerated or retarded by these 
influences. In an epileptic, who has been free from attacks 
for years, a seizure may suddenly develop in consequence of 
fright ; indeed, a person who has been apparently well up to 
that time may have an epileptic seizure in consequence of fright 
and the disease may then continue for the rest of his life. Such 
a condition seems only possible in individuals who are predis- 
posed to the disease, and in whom it only needs a slight stimu- 
lus to produce the attack. The fright is the drop which causes 
the full vessel to overflow, but which in an empty vessel would 
make no difference ; a sound person never becomes an epilep- 
tic owing to fright. 

In the second place we have traumatisms and more espe- 
cially injuries to any portion of the head. It may happen that a 
person previously perfectly well is taken with an epileptic fit 



574 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

after a fall or blow upon the head and post mortem not the 
slightest changes can be detected in the brain. In such cases 
we should always carefully examine the skull and overlook no 
scar, however trivial, because any one may be the cause of the 
first epileptic attack. If this is the case we have the so-called 
" reflex epilepsy," which has already been mentioned, and which 
in the stricter sense is not genuine epilepsy. Reflex attacks 
may also be determined by painful cicatrices on the peripheral 
nerves on any part of the body, or by the existence of ulcer- 
ative processes, for instance, of the finger nails. In one of my 
patients it was possible every time to produce an attack by 
pressure upon the diseased matrix of the nail, the same thing 
occurring also when he accidentally struck it against anything. 
The amputation of the terminal phalanx was followed by com- 
plete recovery after all other measures had proved fruitless. 
In a similar manner polypi of the ear (" ear epilepsy "), inflam- 
matory processes in the ear, intestinal parasites, an incarcerated 
hernia, and lastly diseases of the sexual organs, in the male as 
well as in the female, may give rise to epileptic attacks. Fur- 
ther, we must mention the influence of the imitative impulse 
upon the occurrence of epileptic attacks. If nervous individu- 
als frequently see epileptiform convulsions it may happen that 
they succumb to them themselves. In the royal prison of 
Breslau I have known thirteen of a large number of female 
inmates who were working together in a room to become epi- 
leptic a short time after another prisoner, who had been suf- 
fering from epilepsy for years, had been brought into the same 
ward. 

I have reported the occurrence of an epidemic in a school 
(Berliner klin. Wochensch., 1893, 50). Bad air, especially in 
cafe's, where there is a good deal of tobacco smoke and poor 
ventilation, predisposes the epileptic to attacks, especially if 
loud talking or music is going on. The mental excitement 
produced by such stimuli may precipitate an attack. Epilep- 
tics should be warned not to go to dances, since the many dif- 
ferent factors which are here combined may aid in producing 
an attack. 

The manner in which an epileptic patient can spend his life, 
the possibility of doing justice to the requirements of his call- 
ing and of being a more or less useful member of society, the 
outlook for improvement or even recovery — all these questions 
depend in the main upon the " attacks " to which he is subject, 



EPILEPSY. 



575 



on their nature, their duration, their frequency, their after- 
effects, and so forth. Hence it is our first duty in taking 
charge of a case of epilepsy to study carefully the attack itself. 

Symptomatology. — The " Attack." — There are cases in 
which the attack occurs suddenly and unexpectedly, so that 
the patient, until now in apparently perfect health, falls to the 
ground as if struck by lightning. In others — more numerous — 
it is announced, so to speak, by certain premonitions, which, to 
maintain Galen's old expression, we call auras. 

In the study even of the aura we can not help being struck 
with the fact, which, on a closer examination of the attack, is 
still more impressed upon us, that no two cases of epilepsy are 
alike, that almost every one has its own peculiarities, so that a 
comprehensive description is almost impossible. The premo- 
nitions are countless and many attempts have been made to di- 
vide them into classes. Even if we have obtained a classifica- 
tion we are far from possessing with it a description of all. 

First of all, we may subdivide the auras into psychical and 
somatic. In the former case the patient may either become 
surprisingly quiet and look meditative, or he may present 
signs of excitement, walk anxiously up and down the room, 
and seem bewildered. The transition from the aura to the 
actual pre-epileptic disturbance of consciousness, the pre-epilep- 
tic insanity, is not appreciable (Mendel, Eulenberg's Viertel- 
jahrschrift, N. F., 1885, Bd. 42, Heft 2). This prodromal state 
may extend over several hours, although it may not last 
longer than thirty seconds or a few minutes. In two cases 
the patients told me that, immediately before the attack, 
reminiscences of bygone days forced themselves upon their 
minds, and that portions of their past lives rapidly passed 
before them. A psychical aura of this kind is rare. Some- 
times an irresistible desire in the patient to run away constitutes 
the aura. Just as we shall see in the form of epilepsy called 
epilepsia procursiva, the patient escapes from his home and 
runs great distances. While he is running he is seized with 
the attack. Midway between the cases in which there is a 
psychical and those in which there is a somatic aura come 
those instances in which the patient complains of vertigo, 
violent headache, and slight disturbances of consciousness, 
symptoms which may last but a very short time, and which, 
indeed, may be of such brief duration that the patient 
has not time to guard himself against falling. Here, too, be- 



576 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

long the hallucinations which occur in the domain of the 
nerves of special sense, which we are accustomed to call 
" special sense" auras. The patient hears, sees, smells, tastes 
things which either are not there at all or are in reality differ- 
ent from what he deems them. I know instances in which im- 
mediately before the fit the patient thinks he is standing in a 
sea of light ; most intense brightness surrounds him, and he is 
cognizant of wonderful light effects. In other cases again the 
patient thinks he is standing amid utter darkness, he sees noth- 
ing, and the densest obscurity reigns everywhere. To this 
class belong the instances reported by Heinemann in which 
bilateral amauroses constituted the aura (Virchow's Arch., 
102, 3, 1885, p. 522). The optic as well as the auditory auras 
vary in different patients. Sometimes they hear delightful 
melodies, sometimes they find themselves amid the wildest tu- 
mult of confused noises. Complete loss of hearing, transient 
deafness, which would be analogous to the transient amauro- 
sis, I have never had an opportunity to note. 

Sometimes, not often, the patients imagine they hear dis- 
tinctly different voices, Then the aura is a genuine hallucina- 
tion and infringes upon the domain of pre-epileptic insanity. 
Well-pronounced gustatory and olfactory auras do occur, but 
are decidedly less frequent than those just described. 

The somatic auras are either motor, sensory, or vaso-motor. 
The motor more frequently consist of symptoms of irritation 
than of paralysis. There are isolated twitchings in the fin- 
gers or toes, in the arms or legs, which progress from the 
periphery to the centre ; contractures in certain fingers have 
also been observed. In addition to or in the place of these 
there may be twitching movements of the head or neck, twitch- 
ings of the facial muscles, or well-marked strabismus. Paretic 
symptoms, heaviness and fatigue in the extremities, are more 
rare. Spasm of the glottis, bronchial asthma, palpitation of 
the heart, retching — all have to be regarded as varieties of 
motor auras. 

The sensory auras consist of peculiar parassthesias in the 
extremities, formication, numbness in the fingers, the patient 
feeling as if these were working their way up to the head or to 
the heart. Not uncommonly they are associated with a pro- 
nounced feeling of anxiety and oppression. The sensations 
which appear in the extremities, sometimes in the fingers, 
sometimes in the toes, are 'extremely variable, from a pleasant 



EPILEPSY. ^jj 

slight tingling to a painful burning and stinging, which, as we 
have said, proceeds from the periphery to the centre. 

In vaso-motor aurse the hands become cold and pale, the 
cutaneous veins look less full than normally, and the patient 
complains that he is getting cold. A general feeling of chilli- 
ness, associated with chattering of the teeth, has also been 
noted (Douty, Lancet, March 20, 1886). In other instances, 
possibly on account of a paralysis of the vaso-motor nerves, 
blushing of the skin and sweating occur. The degree of full- 
ness of the cutaneous vessels and the larger veins of the skin 
is in some cases sufficient to tell the patient whether or not he 
will shortly have a fit. 

Innumerable transition forms and countless combinations of 
different kinds of auras occur. No definite laws can be given, 
and we must here again recall the inexhaustible varieties of the 
prodromes by which the attack may be ushered in. 

The question whether the origin of the aura be central or 
peripheral can not as yet be answered. Certain facts point to 
the first possibility, others to the second (cf. Oliver, Lancet, 
April 21, 1888, page 769). That the aura may have an anatom- 
ical basis is proved by the case reported by Hughlings Jack- 
son (Brit. Med. Journal, February 23, 1888). The patient, a 
man of fifty-three years of age, complained regularly of a hor- 
rible, indescribable stench which immediately preceded every 
attack. At the autopsy a tumor was found situated in the 
temporo-sphenoidal region. We would remark, by the way, 
that this case is a point in favor of Ferrier's localization of the 
sense of smell. 

The attack itself is characterized by complete loss of con- 
sciousness, and is sometimes ushered in by an initial piercing 
cry or a noise like the roar of a wild beast which the patients 
emit at the moment of falling. This cry is by no means to be 
regarded as the expression of fear or surprise, as it does not 
occur until consciousness is lost and is a reflex act. It is ob- 
served in about fifty per cent of all cases, while in the remain- 
der it is either absent or replaced by tears. A tonic muscular 
contraction accompanies the cry. The head is at the moment 
of the fall drawn backward or to one side, the jaws are pressed 
together, the back is spasmodically curved, and the fingers are 
clenched over the adducted and flexed thumb. Respiration 
ceases, because the muscles performing the function take part 
in the spasm, and the face becomes discolored and cyanotic. 

37 



578 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

A convulsive tremor runs over the whole body, and in the 
muscles of the face as well as in the rigid extremities twitch- 
ings begin to appear, which spread, and spare no part of the 
body. The head is violently knocked against the floor or the 
couch, the tongue rolled around in the mouth, protruded, per- 
haps, and retracted alternately, so that it is often injured by 
the teeth ; the eyeballs are deviated, the pupils dilated and in- 
active. Arms, legs, and trunk are now the seat of violent, ir- 
regular, rapidly changing jerkings. The mechanism of these 
motions has been studied by Unverricht (Ueber tonische und 
klonische Muskelkrampfe, Leipzig, 1890). Corneal and skin 
reflexes are lost. The tendon reflexes can be obtained if the 
tetanic rigidity of the extremities allows it. The pulse is 
slightly quicker, the respiration greatly hurried. With each 
expiration the saliva, often foaming and mixed with the blood 
coming from the injured tongue, bursts forth and covers the 
lips. The temperature remains normal. In more protracted 
cases it may rise from one fifth to half a degree Fahrenheit. 
The involuntary evacuation of urine and fasces, possibly also of 
semen, is not rare. In one case only have I seen the attack 
regularly associated at its onset with vomiting. 

Gradually the body becomes covered with sweat in conse- 
quence of the excessive muscular strain ; next, the convulsions 
lose some of their violence, the limbs gradually become less 
rigid, the cyanosis disappears, respiration, though it may still 
be difficult and snoring, becomes more regular, the coma 
abates and passes insensibly either into a deep, long sleep or 
gives place immediately to complete consciousness, so that in 
some cases the patient may in a few minutes again be in an 
apparently perfectly normal condition, without, however, hav- 
ing the slightest idea of what has been going on during the 
attack. 

We have said that the symptoms immediately preceding 
the attack present an endless variety of forms ; the same must 
be said of those that belong to the period following it. These 
"post-epileptic" phenomena may again be divided into psychi- 
cal and somatic. The psychical phenomena are very interest- 
ing, because they are not always of the same intensity, but 
may assume all gradations between a complete insanity (" post- 
epileptic insanity," post-epileptic moria, Samt) and a slight 
bewilderment. In the first case the patient has to be regarded 
as a madman, and must not be held responsible for his actions, 



EPILEPSY. 



579 



not excluding any crime that he may commit at such times ; 
in the latter he resembles a drunken man, who, although he 
later can not remember what has happened, will answer ques- 
tions if they are repeated often enough and in a sufficiently 
loud tone. Not uncommonly there exist on first waking up 
speech disturbances, in the form of a motor or sensory aphasia, 
which lasts from a few minutes to several hours. Total apha- 
sia following the attack has also come under my notice, and I 
have seen it persist for half an hour. The patient appeared to 
have regained consciousness pretty well, he understood, appar- 
ently, the questions which were asked him, but was not able to 
answer them in any other way than by signs. Fiirstner has re- 
ported instances of post-epileptic stammering (Arch. f. Psych, 
und Nervenkrankheiten, 1886, xvii, 2). 

Among the somatic post-epileptic phenomena there is, be- 
sides the difference in the size of the pupils, which is of some 
value for the diagnosis of nocturnal attacks occurring during 
sleep, a concentric contraction of the field of vision, which may 
last for twenty-four hours. Of this I have been able to con- 
vince myself several times positively. Further, there are cer- 
tain conditions of motor irritation, ''cortical movements" 
(Rindenbewegungen of Zacher), which consist of either typi- 
cal clonic twitchings, or of choreoid or athetoid movements, 
and which may persist for hours. Contractures, occurring 
more frequently in the upper than in the lower extremities, 
usually on one side, have been observed only in exceptional 
cases (Lemoine, Deutsche Med.-Ztg., 1888, 20). Among the 
vaso-motor changes there are circumscribed reddenings which 
may occur symmetrically on both sides of the body in the 
most diverse places. Transient increase of the patellar reflex, 
transient albuminuria and violent vomiting are common after 
epileptic attacks. 

As to the time at which the attack may be expected, we 
may broadly say that there is not a moment in the life of the 
patient in which he can feel safe from them ; that any particu- 
lar time, either of the day or of the night, is especially danger- 
ous in this regard can not be maintained. This much only can 
be said, that in some individual cases the fits occur only during 
the night while the patient is in bed and asleep ; this so-called 
epilepsia nocturna possesses great practical importance, be- 
cause it may persist for a very long time unremarked and un- 
recognized, especially if the patient sleeps alone. If such be 



580 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

the case, the diagnosis can only be made from certain charac- 
teristic signs observed in the morning — from the pain of the 
bitten tongue, the dull headache, the slight extravasation of 
blood into the conjunctivae, or the unequal pupils (vide supra). 
In one of ray cases of nocturnal epilepsy there occurs after each 
attack a deep-red spot, the size of the palm of the hand, on the 
forehead, which does not begin to fade until one or two days 
have passed. For years the attacks may be confined to the 
night, and may go on without interfering to any extent with 
the patient's business and social life. Above all, he is not ex- 
posed to the usual injuries caused by the falls, but he never 
can feel absolutely certain that some time or other an attack 
may not occur during the dav. These nocturnal fits are 
heralded by an irregular respiration, snoring, grunting, or 
moaning. Convulsions may not occur at all, but the whole 
body gets into a condition of tetanic rigidity which is followed 
by a relaxation of the muscles; during the whole time the 
patient does not awake, and has no consciousness of what has 
been going on. 

There are certain things which seem to exert an unfavor- 
able influence upon the severity and the frequency of the fits, 
and against which the patient must be strictly and repeatedly 
cautioned. These have been mentioned on page 573. It need 
only be added here that coitus does not always have a bad in- 
fluence, and that there is no reason, from the physician's point 
of view, for forbidding it altogether. Whether the climate has 
anything to do with the fits we are not sure, and the idea of 
the supposed influence of the moon must be relegated to the 
domain of the unknown. It is interesting to note, however, 
that when an epileptic is taken ill with typhoid fever, pneumo- 
nia, facial neuralgia, etc., he may hope to enjoy immunity from 
the attacks as long as these diseases last. This, however, does 
not hold good for pregnancy ; according to Nerlinger, to whom 
we owe an interesting monograph on the relation between 
child-bearing and epilepsy (Heidelberg, Winter, 1889), a dimi- 
nution of the attacks during gestation is observed only in rare 
instances. 

On the other hand, there are certain things which exert a 
favorable influence, either by aborting or preventing for cer- 
tain periods the occurrence of the attacks. How these factors 
work is quite inexplicable. Among the former may be men- 
tioned the application of a tight bandage or strap to the part 



EPILEPSY. 



5 8l 



of the body — e. g., the finger or hand — in which the motor 
aura occurs ; to the latter belongs frequent epistaxis, as I have 
repeatedly had occasion to observe ; if it was profuse it seemed 
to produce an intermission in the occurrence of the attacks 
which lasted for a relatively long time. 

Besides the classical attack which we have just described, 
and which is known as " grand mal" there occurs the rudimen- 
tary abortive attack, as it were, which has received the name 
"petit mat" Of this latter kind there exist countless varieties. 
There may be nothing more than a momentary vertigo, with- 
out any loss of consciousness; this is termed epileptic vertigo; 
or in place of or following this there may be a brief loss of 
consciousness, lasting but a few seconds, the "absence" of the 
French writers, of the onset and the duration of which the 
patient is unable to give any account. An individual may in 
the middle of any kind of occupation — speaking, eating, read- 
ing, and so forth — suddenly stop what he is doing ; for an in- 
stant he stares vacantly before him, remains as he is, standing 
or sitting, and immediately after the " attack " resumes his oc- 
cupation as if nothing had happened ; the unfinished sentence 
is after a short pause completed, the spoon which was ready 
to bring the food to the mouth, after a short stop reaches its 
goal. If an "absence " occurs to the patient on the street when 
he is out walking, he keeps on mechanically, loses his way per- 
haps, and only finds it again when consciousness returns. The 
instances in which such periods take in a much longer time, 
during which the patients undertake voyages, spend money, 
transact business of which they are not conscious later, or do 
things which are against their intention and entail disagreeable 
consequences, must also be looked upon as coming under the 
head of epilepsy. They are undoubtedly rare, and up to this 
time have been carefully observed only by French physicians, 
more especially by Charcot (" automatisme ambtilatoire "). Insig- 
nificant as petit mal may seem, it often has a very deleterious 
effect upon the general condition of the patient, especially upon 
the mind ; we should be cautious, therefore, with our prognosis. 

There are still other seizures in which typical convulsions 
do not occur, but in which the patient suddenly begins to 
walk first forward, then backward, to run around in a circle 
(" mouvements de manege"'), or spin round and round; or he 
may rush out of his house and run for long distances without 
knowing why or whither. This form, which has been de- 



^ 82 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

scribed by Bourneville, Ladame, Weinstock (Inaugur.-Dissert., 
Berlin, 1889), and others, is called "running epilepsy," epi- 
lepsia procursiva. It often appears in childhood, and later 
gives place to the usual classical attacks. Its frequent com- 
bination with moral insanity is interesting. Anatomical changes 
have not been found in the cases which came to autopsy up to 
the present (cf. Biittner, Allg. Zeitschr. f. Psychiatrie, 1891, 
xlvii, Heft 5). 

Again, instead of the convulsive attacks, we may have from 
time to time transient psychical disturbances, which consist 
of states of excitement or depression ; in such instances we 
speak of " epileptic equivalents " (Samt). We must leave to 
the psychiatrists the task of investigating their cause and their 
significance. From a medico-legal point of view these puzzling- 
conditions possess great interest. 

About the frequency of the paroxysms no definite state- 
ment is possible. There are people who during their whole 
life have not more than one, two, three, six, or ten attacks, and 
again there are others in whom they recur once a week or still 
more frequently. Sometimes there are certain periods in 
which they increase in frequency, and others of months or 
years during which only an occasional attack occurs. In rare 
instances, in periods of the former kind, the fits may succeed 
each other so closely that there may be one or even many 
every day. Before the patient has had time to regain his full 
consciousness another attack looms up. This is what we call 
the status epilepticus, e'tat de mal. The temperature may rise 
steadily for from three to eight days as much as 5 to J° F., so 
that it may reach 104 or 106 F. If, then, in the intervals 
consciousness does not become fully restored, but the patient 
remains dull and bewildered, there is very great danger that 
death may occur during the status epilepticus, and the friends 
should be made acquainted with the seriousness of the situa- 
tion. Only in exceptional cases does recovery take place and 
the temperature fall to normal again (Witkowski, Ueber epi- 
leptisches Fieber u. s. w., Berliner klin. Wochenschr., 1886, 
xxxiii, 43, 44). 

Course. — The course of the disease, the general condition 
of the patient in the intervals between the attacks, the influ- 
ence of the attacks upon the mind and body — all these may 
present great variations. 

The course is very chronic and the disease lasts in most 



EPILEPSY 



583 



cases years and tens of years. Frequently the patient is sub- 
ject to the affection during his whole life. The earlier the first 
attacks make their appearance the less chance is there of their 
complete disappearance. In some cases of " late epilepsy," 
" epilepsie tardive" in which the affection does not begin until 
late in life, it may happen that the attacks completely cease as 
unexpectedly as they came on. Still, a course so favorable as 
this is rare and can never be predicted with certainty. Mendel 
has pointed out that this late form runs in general a milder 
course, and that the mind is less likely to become affected in 
these cases. If the disease has set in in early childhood, the 
influence of the period of puberty is generally very marked. 
The attacks become more frequent, and in women the increase 
in number is observed every month at the time of the menses 
until the time of the menopause. Pregnancy has little influ- 
ence on the attacks, according to my own experience ; some- 
times it appeared as if shortly after conception the number of 
fits was considerably lessened, while in other women there 
seemed to be no change. 

The general condition in the intervals between the attacks 
is by no means the same in all cases. In some, fortunately not 
rare cases, the paroxysms do not cause any bad effects for 
years and nothing morbid can be discovered. The mental fac- 
ulties develop normally or, if already developed, remain good. 
The disposition is cheerful, social intercourse is enjoyed, as 
there is nothing in the bodily condition to interfere with such 
pleasures. The presence of epilepsy does not necessarily pre- 
vent the full development of a genius, as is proved by the uni- 
versally quoted historical examples of Caesar, Alexander the 
Great, Rousseau, Napoleon I, and others. 

In other instances the general condition in the intervals 
leaves much to be desired, and as a rule it is the psychical part 
of the man which suffers most unpleasantly. Either the dispo- 
sition of the patient is changed for the worse, so that he is 
easily excited, irascible, suspicious, peevish, unsociable, and 
disagreeable to those around him, or the mental faculties 
suffer, he becomes dull, slow in grasping ideas, indifferent, anx- 
ious, abstracted, and so unreliable in his work that he is no 
longer able to fulfill his duties as a man of business and as a 
good citizen. 

In such cases we are sometimes able to note bodily defects, 
as, for example, abnormities in the formation of the skull, in 



584 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

the form of the auricle, in the condition and arrangement of 
the teeth, and quite frequently flat-foot (Fere et Demantka, 
Journal de l'Anat. et de la Physiol., 1891, 5). Such " signs of de- 
generation," however, are often absent. 

The final issue of the disease is almost always the same. 
The patient remains an epileptic all his life, from time to time 
having attacks, and finally dies from some intercurrent malady. 
The mental faculties may remain throughout, on the whole, 
good and the capacity of the patient for following his calling 
be retained. In other instances the mind becomes gradually 
impaired, so as to necessitate the transference of the patient to 
an institution, or again, in very exceptional cases, there may be 
complete recovery or, at any rate, so marked a decrease in the 
frequency of the attacks that the patient may well regard him- 
self as cured. This cure may come about spontaneously or 
may be caused by some unexpected psychical emotion, par- 
ticularly a fright. However, we should beware of being too 
precipitate in calling a patient " well," because now and then 
even after intermissions of years an attack may again make its 
appearance. 

Death rarely ever occurs during an attack, but indirectly 
the paroxysms may cause a fatal issue. The patient during 
a fit may receive serious injuries; he may fall upon his face 
and be suffocated, or fall into the water and be drowned. The 
average life of epileptics is considerably shorter than that of 
other persons. 

Pathogenesis. — The pathogenesis of the epileptic attack is 
totally obscure ; although we know from the experiments of 
Kussmaul and Tenner that the source of the attacks must be 
sought for in the brain, the exact seat of the disease is not known. 
Since the work of Schroder van der Kolk special attention has 
been given to the medulla oblongata, and the discovery by 
Nothnagel of a " spasm centre " in the pons seemed to afford 
much support to the " bulbar theory," but of late years this has 
fallen more and more into discredit, and it is now the brain cor- 
tex which is regarded as the starting point of the convulsions 
(Hitzig, Albertoni, Franck et Pitres, P. Rosenbach). For a long 
time the motor area was thought to be the only region con- 
cerned, but recently Unverricht, who, with his convincing ex- 
periments on animals, has proved himself the most successful 
defender of the cortical theory (after extirpation of an area in 



EPILEPSY. 585 

the cortex he found that he could not obtain spasms in the 
muscle groups corresponding to it) has shown that excitation 
of the posterior cortical regions is also capable of producing 
an attack, hence that these too possess epileptogenic proper- 
ties, and that irritation of the same may by extension of the 
stimulus to the motor area give rise to general convulsions 
(Deutsch. Archiv f. klin. Med., 1888. 44, 1). 

Binswanger agrees that in the lateral portions of the floor 
of the fourth ventricle there are points the stimulation of 
which gives rise to spasms, which, however, he considers to 
be of a reflex nature, and assumes the reflex centres to be sit- 
uated in the dorsal half of the pons. According to his opinion, 
these represent, as it were, a collecting station for the centres 
of the spinal cord, and can not, in the physiological sense, be 
termed " spasm centres." He maintains that we never can 
succeed by electrical or mechanical stimulation of the pons in 
producing real epileptic attacks (Arch. f. Psych, u. Nervenkh., 
1888, xix, 3). 

However probable an association of the cortex with the 
appearance of symptoms of motor irritation may seem, such 
an association is far from explaining the increased salivary 
secretion, the involuntary evacuation of the bladder, the in- 
crease in the frequency of the respirations, etc., and we must 
for the present leave the question open whether or not such 
phenomena depend upon some influence acting on certain cen- 
tres in the brain and spinal cord, the situation and function of 
which we do not as yet know. The question raised by Ziehen 
as to the significance of the subcortical ganglia in the causa- 
tion of an epileptic attack deserves to be looked into more 
closely ; for the present only this seems certain, viz., that (in 
dogs) the clonic part of the convulsive movements produced 
by stimulation of the cortex is connected with the cortex itself, 
while the tonic and the running movements seem to be of sub- 
cortical origin (XIII. Wanderversammlung sliddeutscher Neu- 
rologen. Archiv f. Psych., 1889. xx, 3, p. 584). The possibil- 
ity can not be excluded that in man, as in animals, both regions, 
the cortex as well as the bulb, may be responsible for the 
attack. 

In the second place we are entirely ignorant of the cause of 
the attack ; it is unlikely that a palpable anatomical alteration 
exists, and the claim of Chaslin (Note sur l'anatomie patholo- 
gique de l'epilepsie, dite essentielle, Journal des Connaiss. med., 



586 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

1889, 5 s. x, 12), that a gliosis, which he designates a "sclerose 
nevroglique," is to be regarded as the cause of epilepsy, is by 
no means proved. Much more plausible is the theory that the 
amount of blood in the brain is of importance in this connection, 
but the different writers have never been able to agree whether 
an increase or a diminution in the amount of blood is the cause. 
Many clinical observations speak in favor of ansemia: thus 
Leyden has seen epileptic attacks in cases of aortic stenosis 
undoubtedly as the result of a temporarily insufficient blood 
supply ; Sommer noted their occurrence in a case of anchylosis 
of the atlas which had produced narrowing of the vertebral 
canal in its upper portion (Virchow's Arch., 1890, cxix, Heft 2, 
p. 362). Results pointing in the same direction have been ob- 
tained by Sutnikow in his experimental studies on hyperasmia 
and anaemia of the brain and its relation to epilepsy (Pfluger's 
Arch., 1892, xc, p. 609). On the other hand, Bechterew, whose 
opinion is based on experiments of Todorsky, holds that during 
the attack there occurs an increased blood-flow to the brain 
and a dilatation of the capillaries, and that this condition is the 
cause of the attack. We see, therefore, that the question is by 
no means decided ; we should also think of the possibility that 
vaso-motor changes, or a rapidly or gradually developing 
autointoxication, perhaps by ptomaines (Benedikt) may pro- 
duce the attack. Since epileptic attacks are also sure to occur 
after acute infectious diseases (influenza, typhoid fever), also 
after vaccination (Althaus), an infectious origin can not be ex- 
cluded. But whatever may eventually be shown to be the 
cause, a hereditary abnormal excitability of the psychomotor 
centres has to be regarded as a conditio sine qua non. 

A peculiar kind of epilepsy, which is said only to occur in 
heart disease, has been described by Lemoine (De l'epilepsie 
d'origine cardiaque, Revue de med., vii, May 5, 1877) ; yet since 
the connection is not absolutely proved, and since, moreover, 
the attacks themselves presented no peculiarities of their 
own, we shall limit ourselves to saying that they disappeared 
under the administration of digitalis. 

Von Jaksch (Zeitschr. f. klin. Med., 1885, x, 4) has shown 
that epileptic attacks may be produced by auto-intoxication, 
not only by urea, but in a similar way also by acetone. In 
cases of " epilepsia acetonica " large amounts of acetone were 
found in the urine, which besides contained neither sugar nor 
albumen. The physiological connection between the occur. 



EPILEPSY. 



587 



rence of acetone in large quantities in the urine and epilepti- 
form attacks is not as yet fully established, nor do we know 
how poisons — for instance, lead — introduced into the organism 
from outside are able to produce such attacks ; as a matter 
of fact, however, lead workers suffer so frequently from 
epilepsy that we are justified in assuming the existence of a 
definite " epilepsia saturnina " (Hirt, Krankheiten der Arbeiter, 
iii, 49). 

Briefly, epileptic attacks may occur as a symptom also in 
meningitis, dementia paralytica, during delirium tremens, in 
sclerotic processes, more especially in sclerosis of the cornu 
Ammonis. They may be associated with tumors, hydro- 
cephalus, or abscess of the brain, in which cases they are the 
result of the increased intracranial pressure, as we have 
pointed out above. From what has been said in this and in 
previous chapters it will be understood that these and the so- 
called epileptiform attacks above mentioned have in all proba- 
bility nothing to do with the genuine classical epilepsy. 

Diagnosis. — We can well understand, then, how cautious 
we must be in our diagnosis. Only after repeated and careful 
examinations, after which we are able to exclude organic brain 
diseases, abnormities in metabolism, in consequence of which 
abnormal or poisonous substances occur in the urine (urea, 
sugar, acetone), are we justified in making the diagnosis of 
genuine epilepsy. The skin and tendon reflexes should always 
be carefully examined. Sometimes, from the absence of the 
abdominal or cremasteric reflex, or from a unilateral increase 
of the patellar reflex, we may be able to diagnosticate an 
organic brain trouble when we otherwise, without any inquiry 
into the condition of the reflexes, might have regarded the case 
as one of genuine epilepsy. 

Quite frequently we meet with malingerers who, for some 
reason or other, feign epilepsy. The situations in which the 
simulation of this disease would be likely to be advantageous 
to the deceiver are quite numerous, and it would be impossible 
to enter into the consideration of them here ; we will only men- 
tion that epileptics are exempted from military service, good 
grounds enough for many to sham this disease. The more 
cunning the malingerer the more perfect will be the attack, 
not excluding the foaming at the mouth (made by soap) and 
the (not very deep) wounds of the tongue ; there will be con- 



588 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

vulsions, and the (feigned) loss of consciousness is possibly pro- 
longed more than is necessary ; if the rogue has courage 
enough he will not betray himself either by a reflex motion of 
defense, or even by the slightest twitching, if hot sealing wax 
is, as a test, dropped on his chest. Under certain circum- 
stances it may be extremely difficult to unmask the fraud; it 
might, indeed, be impossible, did we not know one reflex over 
which the will has no power, namely, the pupillary reaction to 
light, which in the epileptic is lost, in the malingerer naturally 
is retained. In doubtful cases, therefore, this reflex has to be 
carefully observed, and the further measures should depend 
upon its condition. 

Treatment. — The treatment of epilepsy confirms the old 
experience that the greater the number of remedies which 
become known and are recommended for a disease, the more 
difficult and uncertain becomes the cure. In the course of 
centuries such an array of medicaments have been recom- 
mended to combat this disease that there is hardly a drug in 
the shops which has not at one time or another been regarded 
and praised as an infallible ''specific." Unfortunately, all these 
claims have been proved to be false. We are to-day as little 
in a position to cure epilepsy as we were one or five centuries 
ago. Only by the discovery of some causes which may pro- 
duce epilepsy, the removal of which lies in our power, has any 
progress been made in the treatment of the disease. This more 
particularly applies to the above-mentioned reflex epilepsies, 
and the Jacksonian variety, which, it is true, is not a genuine 
epilepsy. Here a cure is possible — nay, we may say even cer- 
tain — if we are able to remove the cause. To discover it must 
be the physician's aim. Sometimes it consists of a bone splinter 
which has been left after an injury to irritate the cortex, in 
which case a cure will invariably be effected by the operation 
of trephining for the removal of the splinter. The principles 
which should guide us in such an operation, the foremost of 
which is to make as large an opening as possible, have been 
formulated, among others, by V. Horsley at the French Con- 
gress for Surgery (Wien Med. Presse, 1891, 16). In other in- 
stances painful cicatrices have to be excised or affections of 
the intestinal tract or the sexual apparatus treated. In children 
the natural openings of the body have to be examined for the 
possible presence of a foreign substance, the removal of which 
would then be absolutely necessary. 



EPILEPSY. 



589 



Such are the favorable cases in which it is in the power 
of the physician to bring" about a cure. Unfortunately, their 
number is not great. In the largest majority of instances we 
are not able to find any cause, the removal of which would re- 
move also the disease ; but to-day, as centuries ago, we are re- 
duced to the sad necessity of trying all sorts of remedies, trust- 
ing to good luck that at some time we may hit upon one which 
is truly efficacious. Before relying upon the action of any 
drug, or together with the administration of the remedy chosen, 
strict attention should be paid to the condition of the stomach ; 
indigestion should be prevented, or if it exists should at once 
be treated, if necessary by emptying the stomach with the 
tube (Alt, Munch, med. YVochenschr., 1894, 14). The fact that 
I have observed the occurrence of attacks to be more frequent 
when much food was given which was rich in nitrogen, has 
prompted me to limit the use of nitrogenous articles of food 
and to advise total abstinence from meat at least three days in 
the week. Some epileptics have improved their condition 
considerably by becoming vegetarians ; whether they ever re- 
cover absolutely under that regimen I am unable as yet to 
decide. To counteract any intestinal sepsis Fere recommends 
naphthol and salicylate of bismuth. 

Among the internal medicines the so-called specifics possess 
an interest purely historical ; from artemisia (in hot beer, 10 to 
20 grm. at a dose — grs. 1 50 to 300) and Valeriana down to squilla, 
gratiola, sedum, cardamine, and hellebore, many herbs have 
been lauded as effectual. Asafcetida, castoreum, and camphor 
have been recommended, although no better results have been 
obtained from them than from silver nitrate, ammonio-sulphate 
of copper, and arsenic. A great sensation was created by Meg- 
lin's pills, which, in addition to zincum album contained hyos- 
cyamus. Some have sworn by oxide of zinc, and Herpin, for 
instance, claimed that out of forty-two cases he cured twenty- 
eight with it. To unprejudiced judges who continued their 
observations for a sufficiently long time these " cures " could 
not hold their ground. They proved to be deceptive, and we 
were as helpless as before. Recourse was had also to narcot- 
ics, and much was hoped from the action first of opium and 
later of ether and chloroform. It is true that here and there 
an attack has been cut short by inhalations of the latter, but 
that is all. It is not to be wondered at that under such cir- 
cumstances secret remedies were used to a tremendous extent; 



590 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



and to what a pitch the humbug and impudence were car- 
ried may be seen from the composition of some such remedies, 
for instance, the epilepsy powder of the Institute for Deacon- 
esses in Dresden, which consisted of charred bone of magpies 
which had to be shot at some time during the twelve nights 
following Christmas, and again from the epilepsy powder of 
Wepler, which was nothing but charred and pulverized hemp 
thread (cf. Richter, Das Geheimmittelunwesen, Leipzig, 1872, 
pages 15, 16). 

A new era in the treatment of epilepsy — that is, of the at- 
tacks — was initiated when Locock in 1853 recommended bro- 
mide of potassium, which obtained a wide acceptation through 
the efforts of Legrand du Saulle. Its power of diminishing the 
reflex irritability and of lowering the blood pressure in the 
brain has placed it first among the antispasmodics, and to-day 
it has to be regarded as the best and most important medicine 
in the treatment of epilepsy. In order not to be disappointed, 
however, in our expectations, it is necessary that we should be 
familiar with the proper regulation of the dose and with cer- 
tain unpleasant effects which are apt to arise in the course of 
the treatment. The small and moderate doses of 0.5 to 4 grm. 
a day (grs. viij to 3 j) formerly used are generally ineffectual. 
It is necessary to employ much larger amounts, which are 
best given in one dose. It is, moreover, better to combine the 
three bromides, viz., the bromides of potassium, sodium, and 
ammonium, in equal parts than to give bromide of potassium 
alone. The minimum daily dose for adults in cases of pro- 
nounced epilepsy is eight grammes (3 ij), and w r e should fol- 
low Mendel, who advises that it should be taken in valerian 
tea immediately before going to bed (potassium bromide, am- 
monium bromide, aa 2.5 (grs. xxxviij) ; sodii bromidi, 3.0 (grs. 
xiv). For children and young people up to sixteen years of 
age the daily dose should be half a gramme (grs. vij) for every 
year. If the two drachms are not sufficient — that is, if an at- 
tack still occurs now and then — the dose may be increased to 
ten or twelve grammes (3 ijss. to 3 iij), and this continued until 
four or five hundred grammes or from six to nine ounces are 
taken. 

In this way I have treated hundreds of epileptics in private 
as well as in dispensary and hospital practice, and have let slip 
no opportunity for observing the action of the bromides. This 
action is by no means the same in all cases. There are people 



EPILEPSY. 



591 



in whom an idiosyncrasy against the medicine rapidly devel- 
ops, so that it is impossible for them to take it any more. It 
nauseates them and may cause vomiting, and after repeated 
unsuccessful trials to resume the treatment we have to discon- 
tinue it entirely. In other instances the desired effect on the 
attacks may show itself; but after a few weeks the patient be- 
gins to complain of general bodily and mental feebleness, a 
constant desire to sleep, some loss of memory, and other symp- 
toms, so that the dose has to be diminished. At the same time, 
sometimes without these symptoms, an eruption on the skin 
appears, more especially an extensive, obstinate acne distributed 
over face, trunk, and extremities, which is most distressing, 
especially to young female patients. I have seen this eruption 
particularly after the prolonged use of small doses, and have 
also seen it disappear comparatively rapidly under the use of 
mild laxatives and the administration of arsenic in the form of 
Fowler's solution. Finally, cases come under our notice in 
which bromide, no matter in what form or dose it be given, is 
entirely without effect. The attacks occur just as they did 
previous to the administration of it. Here we have, of course, 
again to suspend the treatment, more especially if symptoms of 
intoxication appear in addition to the continuance of the fits. 
If we wish to express the effects of bromide in epilepsy by 
percentages, we could say that in about ninety per cent of all 
cases the paroxysms diminish in number and violence, that in 
about as many signs of bromism appear which render neces- 
sary a diminution of the dose or gradual suspension of the 
medicine. In from two to three per cent of all cases bromide 
is borne so badly that it has very early to be discontinued. 

If it is established beyond doubt that the bromides exert a 
favorable action, we must insist upon their prolonged use for 
months and years. To add some variety to the treatment they 
may be combined with belladonna and pills may be ordered 
which contain both. If every evening two centigrammes (gr. 
1 j of belladonna and two grammes (grs. xxx) of bromide are 
given, about the same results are obtained as with eight 
grammes ( 3 ij) of bromide alone. [I£ Extr. bellad., 0.5 (grs. 
vijss.) ; pot. brom., sodii brom., ammonii brom., aa 15 (§ss. 
circ.) ; pulv. et succ. liq., aa q. s. ut. f. pil. No. 50. Signa: One 
to two pills in the evening.] When the action of the bromide 
gradually becomes lessened owing to the establishment of a 
tolerance, the administration of belladonna is also indicated, 



592 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

and it may then be given in the form of Trousseau's pills. (IJ 
Extr. bell., Fol. bell, aa i.o (grs. xv), succ. q. s. ut. f. pil. No. ioo. 
Signa : One to two, later three to four, or even six pills, in the 
evening.) 

Compared with bromide and belladonna, which, according 
to our opinion, are the only reliable drugs to be used in the in- 
ternal treatment of epilepsy, the medicaments which have been 
recommended of late years — curare (considered to be ineffect- 
ual by Bourneville), anlipyrine by Beaumetz, tinct. simulo (the 
fruit of Capparis coriacea), which has been used by White — do 
not play any important role, and only deserve a trial in desper- 
ate cases. With my trials with borax, which has recently been 
so often recommended, I have been somewhat disappointed. 
On the other hand, amylene hydrate, recommended by Wilder- 
muth (cf. lit.), must be given a trial in cases of distressing bro- 
mism or if the attacks increase to an alarming extent. The 
watery solution of Kahlbaum's preparation, in the proportion 
of one to ten, is the best to use in doses of from twenty to forty 
grammes (3 v to 3 x) — i. e., two to four grammes (3ss. to 3j) 
of the drug itself. It may be given in wine or water or in a 
glass of beer, well shaken up, and from five to eight grammes 
of the drug(3jss. to 3 ij) may thus be used daily. Flechsig 
(Neurol. CentralbL, 1893, 7) has recommended extr. opii. 0.2- 
0.3 p. d. (3-4J grains) for six weeks, followed immediately by 
large doses of the bromides. I have no personal experience 
with this treatment. 

Surgical interference has also been resorted to, at first with 
the view of influencing or diminishing the amount of blood in 
the brain. Several times the carotids have been ligated, and 
two cases thus treated were reported as completely cured 
(Hasse, Krankheiten des Nervensystems, p. 297). Owing to 
the great difficulties of the operation and the grave responsi- 
bility which the physician takes upon himself, this measure 
will only in exceptional cases be made use of. With bleeding, 
strong revulsives to the skin, such as Autenrieth's ointment to 
the shaved head, moxas, setons, blisters, and purgatives, possi- 
bly the same results can be obtained. 

More recently both vertebral arteries have been ligated 
(von Baracz, cf. lit.). In my clinic the ligation of one verte- 
bral, the right, was performed several months ago by Janicke 
without any noticeable effect upon the frequency or the sever- 
ity of the attacks; hence the patient was not willing to submit 



EPILEPSY. 



593 



to the ligation of the other. The operative treatment of trau- 
matic epilepsy aims at the removal of bone splinters which 
press upon and injure the brain cortex ; but the operation 
should only be performed, as von Bergmann holds, if the con- 
vulsions constantly occur in the same groups of muscles and 
extend in a characteristic manner, or if transient hemipareses 
occur. At the operation the affected area of the cortex has to 
be carefully excised. If the attack begins like a flash without 
an aura and is associated with opisthotonus, etc., operative 
measures are contraindicated. Neurotomy of the sympathetic, 
a procedure described by von Jaksch (Wien med. Wochen- 
schrift, 1892, 16, 17), has produced a cessation of the attacks for 
several months in a number of instances ; but we do not know 
whether it is capable of bringing about a permanent cure. 

Marshall Hall's advice to perform tracheotomy, on the 
ground that the spasm of the glottis is productive of the as- 
phyxia and the clonic spasm, is purely and entirely of histor- 
ical interest. The operation has been performed several times 
without, of course, the least benefit to the patient. The same 
may be said of the cauterization of the glottis with nitrate 
of silver, suggested by Brown-Sequard, which has been justly 
condemned in such cases. 

In connection with the surgical treatment we should men- 
tion the application of strips of cantharidal plaster around the 
forearm or lower leg in which the motor or sensory aura oc- 
curs. Only when the aura constantly appears in the same 
member can any success be expected from this measure, which 
has been recommended by Buzzard. The plasters must re- 
main on for a considerable time. Following the advice of Buz- 
zard, I have ordered the application of these plasters in some 
cases, without, however, having been able to see any good re- 
sults. In one instance of partial epilepsy a transfer was pro- 
duced by the application of the plaster (Hirt, Neurol. Central- 
blatt, 1884, 1). 

Finally, we can hardly be surprised that attempts have been 
made to combat epilepsy by electrical treatment. Unfortu- 
nately, the results with this have been even less encouraging 
than those from internal medication. Neither the attacks them- 
selves nor the so-called " epileptic change in the brain," the 
nature of which, as we have above stated, is still obscure, have 
been influenced by it in any way. The constant current was 
employed and the sympathetic galvanized by passing the cur- 
38 



594 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

rent from one mastoid process to the other, and attempts 
were made to influence the cerebral hemispheres, and more 
especially the motor regions, according to Erb's method 
(Erb, Handbuch der Electrotherapie, p. 581). In other cases 
the current was passed through the lobes of the thyroid 
gland, as Sighicelli (Riv. sperim. di freniatr, 1888, vol. xiii, 3) 
has more recently done, but in none of them could any last- 
ing success be remarked. No better results have been ob- 
tained with the faradic current in all its different modes of 
application. 

Although with all our treatment we are practically power- 
less against the disease, it would be very wrong to assume that 
to the epileptic the physician can be of no use and can not im- 
prove his condition in any way. On the contrary, there is 
hardly another class of patients affected with nervous diseases 
who require so much a physician's advice, and hardly another 
class who have to be so carefully watched by him. Above all, 
attention has to be paid to the general condition. The bowels 
must be kept regular and the skin and muscles stimulated to 
their proper activity by appropriate cold-water treatment and 
home gymnastics. The patient should constantly be warned 
against every kind of excess. Too large a supper, a few glasses 
of wine or beer taken too quickly, any indigestible food, ex- 
cesses in venere — all these may give rise to an attack, the con- 
sequences of which are incalculable. To guard against these, 
therefore — in other words, to employ prophylactic measures — 
is the chief task of the physician who is taking charge of an 
epileptic. Besides this, the bromides, or, if these are not suit- 
able, the next best treatment, should be begun. Finally, care 
must be taken that the patient does not hurt himself during the 
fit, and against this he should be protected as well as possible. 
All tight clothing must be removed and all ordinary emergen- 
cies provided for. A regular treatment of the attack itself 
we do not possess, and all attempts to cut it short should be 
avoided. Even inhalations of amyl nitrite, which Q, Berger 
suggests, chloroform, and similar remedies are only allowable 
if administered with the greatest caution, and it would be bet- 
ter still to discard them entirely. 

Note. — -Eclampsia is one of those terms which up to the 
present do not convey to our minds any clearly defined clinical 
or .pathological picture. It is a term under which are compre- 



ECLAMPSIA. 



595 



hended the most heterogeneous conditions which have not the 
least connection with each other. If a woman during preg- 
nancy or during parturition without any appreciable cause 
loses consciousness and falls into convulsions, which may recur 
several times, and which frequently lead to a fatal issue, we 
speak of eclampsia gravidarum or parturientium. If children, 
as not uncommonly occurs, have paroxysms, consisting of dis- 
tortions of the face, trismus-like clinching of the teeth, general 
spasms, and more or less marked disturbances of consciousness, 
we designate the affection as eclampsia infantum, and use the 
same term if at the onset or in the course of acute diseases or 
certain intoxications (more particularly lead poisoning) attacks 
occur characterized by (bilateral, more rarely unilateral) con- 
vulsions and loss of consciousness, which, therefore, differ clin- 
ically either not at all or only slightly from the genuine epilep- 
tic seizures. The nature of the attacks is as obscure as their 
aetiology. Whether in eclampsia parturientium the diminished 
excretion of urea has to be held responsible for the convulsions, 
and they thus are to be regarded as urasmic, whether in the 
convulsions of children reflex action plays the chief rote, or 
whether we have to deal with autointoxication in which dia- 
cetic acid occurs, in the urine, or whether in all cases the pres- 
ence of a bacillus is necessary (Gerdes, cf. lit.) — all these ques- 
tions have to be left to future investigations. Every one ad- 
mits that, in the second form, dentition, digestive disturbances, 
or intestinal parasites, play a certain part, yet there are cer- 
tainly other factors which deserve consideration in this connec- 
tion — for instance, heredity, a general neuropathic diathesis, 
the health of the parents, and the possible existence of rickets. 
The convulsions of children (eclampsia acuta infantilis) are 
extremely common. Clinically, all cases of this kind are very 
much alike, whereas aetiologically different cases differ greatly. 
In a given case we should, first of all, try to determine whether 
we have to deal with anatomical lesions (of the cortex, etc.), 
or whether these can be excluded ; and only by the most 
careful examination can we avoid errors and are we able 
to make a correct diagnosis. Cortical diseases (cerebral in- 
fantile paralysis), epilepsy, spinal paralysis of children, the 
initial stage of acute diseases, etc., must be taken into con- 
sideration. 

The prognosis is always doubtful, both in adults and in 
children, and the danger is usually greater in pregnant and 



596 diseases of the general nervous system. 

parturient women than in children. Death not rarely occurs 
during the convulsions, as we have said above, and we may 
assume that out of a hundred cases of this kind there are thirty, 
forty, often fifty who die, and the danger increases with the 
duration of the labor and the long continuance of the pains. In 
children a fatal issue is often brought about by a spasm of the 
glottis, rarely by exhaustion. Recovery frequently is incom- 
plete, and there may be left some psychical disturbances, amau- 
rosis or disturbances of speech, etc. 

About the treatment of eclampsia the opinions are even at 
the present time very much divided. In pregnant or parturi- 
ent women cold affusions in a warm bath, as recommended by 
Scanzoni, also the application of large cantharidal plasters to 
the neck, ought to be resorted to as soon as possible ; from the 
nervines we can expect nothing. Mild laxatives, cautious 
venesection, regulation of the functions of diuresis and dia- 
phoresis are in most cases indicated. Often we have no time 
to think of such measures ; in urgent cases Veit (cf. lit.) has 
recommended large doses of morphine, beginning with three 
centigrammes (circ. gr. ss.) and increasing the dose to two or 
three decigrammes (grs. iij-grs. ivss.) a day. The eclampsia of 
children is, according to some — among them Henoch — best 
treated by inhalations of chloroform, which will soon stop the 
convulsions. One ought, they think, to first cut short the con- 
vulsions, and then proceed to find out their cause. Sometimes 
this advice is good, viz., in cases in which there exists no cere- 
bral lesion. If one does exist, or if there are grounds for sus- 
pecting it, the inhalation will prove to be of no use, and may 
rather have a bad effect. It will therefore be necessary to 
attempt to settle this question by as short an examination as 
possible. If we are unable to make up our minds, a tepid bath 
and careful affusions, vinegar enemata, or evaporating lotions, 
etc., to the skin can do no harm. For the beginning this suf- 
fices ; afterward it may be advisable to prescribe ice to the 
head in congestive conditions, possibly even leeches to the 
head, and in cases where collapse seems imminent, vinegar ene- 
mata, strong wine, or injections of ether. The nervines may 
as well be discarded in the treatment of the convulsions, as 
they do no good in this stage ; they may, however, be used later 
when the immediate danger has passed. Wrapping the chil- 
dren in warm moist sheets (after the method of Priessnitz), 
while ice is kept to the head, I have known repeatedly to be 



EPILEPSY. 



597 



effectual. On the whole, even these measures are not reliable, 
and the part which a physician plays in the presence of 
eclampsia of children is by no means enviable. 

LITERATURE. 
I. Epilepsy. 

a. Idiopathic Epilepsy. 

Unverricht. Experimentelle unci klinische Untersuch. liber Epilepsie. Habili- 

tationsschrift, Breslau, 1883. (Contains all the older references.) 
Bourneville, Combarien et Seglas. Recherches clin. et therapeut. sur l'epilep- 

sie, l'hysterie, etc. Paris, 1886, vols. i-vi. 
Erlenmeyer. Die Principien der Epilepsiebehandlung. Wiesbaden, 1886. 
Homen. Beitrag zur Lehre von den epileptogenen Zonen. Centralbl. f. Nerven- 

heilk., 1886, No. 6. 
Ozeretzkowsky. Ueber die Alteration der Sensibilitat bei Epileptischen. Med. 

Obosrenie, 1886, 9. 
Unverricht. Ueber experimentelle Epilepsie. Verhandl. des Congresses fiir 

innere Med., Wiesbaden, 1887. 
Leidesdorf. Wiener med. Wochenschr., 1887, 5, 6. (On Epileptic Equivalents.) 
Vetter. Deutsches Arch. f. klin. Med., 1887, Bd. xl, Heft 3, 4. 
v. Bergmann. Die operative Behandlung der traumatischen Epilepsie. Deutsche 

militararztl. Ztg., 1887, xvi, 8. 
Bourneville et Bricon. De l'epilepsie procursive. Arch, de Neurol., Novembre, 

1888, xvi. 
Marie, P. Progr. med., 1888, xv, 43. 

Haig. Neurol. Centralbl., 1888, vii, 5. (Connection of Epilepsy with the Excre- 
tion of Urea.) 
Binswanger. Arch. f. Psych, u. Nervenkrankh., 1888, xix, 3. (Experimental 

Studies on the Pathogenesis of the Attack.) 
Fournier. Gaz. des hop., 1888, lxi, 105. (Epilepsy and Syphilis.) 
Lemoine. Sur la pathogenie de l'epilepsie. Progr. med., 1888, 16. 
Ladame. Ueber procursive Epilepsie. Internat. klin. Rundschau, 1889. 
v. Baracz. Wiener med. Wochenschr., 1889, 7, 8. (Ligation of the Vertebral 

Arteries.) 
Fere. Note sur l'etat des forces et sur le tremblement chez les epileptiques 

apres les attaques. Nouv. iconogr. de la Salpetr., 1889, ii, 1. 
Wigresworth and Bickerton. On a Connection between Epilepsy and Errors of 

Ocular Refraction. Brain, 1889. xliv, p. 468. 
Wildermuth. Amylenhydrat gegen Epilepsie. Neurol. Centralbl., 1889, 15. 
Pechadre. These de Lyon, 1889. 
Zacchi. Lo sperim., 1S90, Jan. 
Hennig. Deutsche med. Wochenschr., 1890, 26. 
Mairet. Progr. med., 1892, 41. (Treatment with Borax.) 
Eulenburg. Ueber den jetzigen Stand der Epilepsisbehandlung. Therap. 

Monatsh., 1892, vi, 11, 12. 
Gerster and Sachs. The Surgical Treatment of Epilepsy. Am. Journ. Med. 

Sci., 1892, November. 



598 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Kiimmel. Deutsche med. Wochenschr., 1892, 23. 

Babes. Ibid., 1893, 12. 

Eloy. La methode de Brown-Sequard. La medication orchidique, thyroidienne, 

pancreatique, capsulaire et cerebrale, les injections d'extraits organiques, la 

transfusion nerveuse. Paris, 1893. 
Poehl. Spermin bei Autointoxication. Berliner klin. Wochenschr., 1893,36. 
Stein. Deutsche med. Wochenschr., 1893, 42. 
Beekhaus. Ueber den Einfluss intercurrenter Krankheiten und physiol. Processe 

auf die Epilepsie. Inaugur.-Dissert., Miinchen, 1893. 
Bourneville et Cornet. Progr. med., 1893, 49» 5°- (Spermin Injections.) 

b. Jacksonian Epilepsy. 

Unger. Wiener med. Blatter, 1886, xi, 40-44. (Jacksonian Epilepsy in Chil- 
dren.) 

Mendel. Ueber Jackson'sche Epilepsie und Psychose. Allgem. Zeitschr. f. 
Psych., 1887, 44, 2. 

Chauffard. De l'uremie convulsive a. forme de l'epilepsie jacksonienne. Arch. 
gener. de med., July, 1887, pp. 5 et seq. 

Bouchard. Les auto-intoxications dans les maladies. Paris, 1887. 

Lloyd. Boston Medical and Surgical Journal, October 15, 1888, cxix. (Cure by 
trephining and incising the Motor Region.) 

Lowenfeld. Ueber Jackson'sche Epilepsie. Miinchener med. Wochenschr., 
1888, xxxv, 48. 

Pitres. Revue de med., 1888, viii, 8. (Clinical Equivalents of Jacksonian Epi- 
lepsy.) 

Jackson, Hughlings. Brain, July, 1888, xi. 

Berbez. Gaz. des hop., 1888, 50. 

II. Eclampsia. 

Lewandowski. Berliner klin. Wochenschr., 1885, xxii, 37. 

Ballantyne. Sphygmographic Tracings in Puerperal Eclampsia. Edinb. Med. 

Journ., May, 1885, xxx, p. 1007. 
Proux. On the Treatment of Eclampsia Infantum. Bull, gener. de therap., May 

15, 1885, cviii. (Recommends belladonna and chloral hydrate.) 
Rosenstein, L. Die Pathologie und Therapie der Nierenkrankheiten. Berlin, 

1886, 3 Aufl. 
Soltmann, O. Eclampsia infantum. Real-Encyclopadie der gesammten Heil- 

kunde. Wien und Leipzig, 1886. 
Virchow, R. Ueber Fettembolie u. Eclampsie. Berliner klin. Wochenschr., 

1886, xxiii, 30. 
Osthoff. Beitrage zur Lehre von der Eclampsie und Uramie. v. Volkmann's 

klin. Vortrage, 1886, 266. 
Stumpf. Munch, med. Wochenschr., 1887, xxxiv, 35, 36. 
Pfannenstiel. Centralbl. f. Gynakol., 1887, xi, 38. (Death from Apoplexy.) 
Baginsky. Archiv f. Kinderheilk., 1887, xi, 1. (Acetonuria in Eclampsia.) 
Veit. Ueber die Behandlung der puerperalen Eclampsie. Volkmann's klin, 

Vortrage, 1887, No. 304. 
Hermann, Ernest. Transactions of the Obstetrical Society of London for the 

year 1887, vol. xxix, pp. 539-548. London, 1888. 



ECLAMPSIA. 



599 



Lantos. Beitrage zur Lehre von der Eclampsie und Albuminuric Arch. f. 

Gynakol., 1888, xxxii, 3, p. 364. 
Feustell. Beitrage zur Pathologie und Therapie der puerperalen Eclampsie. 

Inaug.-Diss. Berlin, 1888. 
Love. Weekly Medical Review, 1880, xix, 1. (Eel. infant.) 
Olshausen. Deutsche Med.-Ztg., 1891, 103; and 1892, 9. 
Herff. Munch, med. Wochenschr., 1891, 5. 
Gerdes. Ueber den Eclampsie-bacillus, etc. Deutsche med. Wochenschr., 

1892, 26. 
Hofmeister. Zur Charakteristik der Eclampsie-bacillus Gerdes'. Fortschr. d. 

Med., 1892, 22, 23. 
Favre. Virchow's Archiv, 1892, exxvii, 1. 
Duhrssen. Arch. f. Gynak., 1892, Heft 3. 
Haegler. Centralbl. f. Gynak., 1892, 51. 
Doderlein. Ibid., 1893, 1. 



CHAPTER IV. 

HYSTERO-EPILEPSY MAJOR HYSTERIA HYPNOTISM TREATMENT BY 

SUGGESTION. 

The reason why we have not treated of the disease, we are 
about to describe, in immediate connection with hysteria, but 
have placed it after the chapter on epilepsy, is because the " at- 
tacks " of hystero-epilepsy appear to the observer as a result, 
or perhaps we had better say as a sort of mixture, of hysteria 
and epilepsy. It would, however, be a mistake to infer from 
this that the affection has any close physiological or patholog- 
ical connection with epilepsy. It is more likely that we ought 
to regard it as a higher, or indeed the highest, grade of hys- 
teria (cf. page 553). 

The " major attacks " have been studied exclusively by 
Charcot in the Salpetriere. To him alone and some of his 
pupils, more particularly P. Richer, we owe our knowledge of 
their nature and characteristics, and of the rules and definite 
laws which they appear to follow. Almost every, nay, we can 
well say every publication on hytero-epilepsy that did not 
emanate from the Salpetriere was, at any rate, based upon 
Charcot's observations and communications, and hardly any- 
thing new has come from any other source. 

The attacks can usually be divided into four distinct peri- 
ods, though one or other of them may so predominate, as re- 
gards its duration and intensity, that the rest are somewhat 
obscured. The first period embraces the epileptiform attacks : 
the body is suddenly shaken, respiration stops, the patient 
lets fall anything she happens to be holding in her hands, and 
is thrown to the ground. She is now seized with general 
convulsions or there develop rapidly extensive contractures 
affecting almost all the voluntary muscles. In the second stage, 
which immediately follows this, the patient is bounced up and 

down in bed, she assumes marvelous positions, stands on her 
600 



HYSTERO-EPILEPSY. 6oi 

head, curves the body in the form of an arch {arc de cercle), 
and howls and roars at the same time like a wild beast. This 
is the period of major movements, " clownism." It is fol- 
lowed immediately by certain hallucinations, under the influ- 
ence of which the patients assume postures indicative of the 
most varied passions, the " attitudes passionelles " of the French. 
The face takes on, according to the particular hallucination, 
an expression of anger, rage, devotion, love, voluptuousness, 
curiosity, pain, etc., which would give us the impression that 
the patient is passing in her mind through a period of her life 
the details of which are unusually vivid in her memory. The 
postures and expressions may change, although sometimes 
they remain the same throughout this stage. That of the 
" crucified " has obtained a certain degree of celebrity, because 
it seems to be particularly frequent. Finally, the fourth stage 
is marked by a delirium, in which hallucinations recur with 
the greatest persistency, some patients imagining they see 
animals, others terror-inspiring objects of different kinds, and 
so forth. Automatic movements are not rare ; sometimes an- 
aesthesias or at least analgesias are noted. This delirium re- 
sembles in many respects an alcoholic intoxication. The dura- 
tion and frequency of the attacks vary greatly. Some only 
last from one to five minutes, and recur ten, twenty, or even 
one hundred times a day {/tat de mat). It is a characteristic 
feature, and one very valuable in the differential diagnosis, that 
firm pressure upon the ovaries invariably suffices to cut short 
an attack. 

If this pressure is exerted constantly, as can be done by means 
of belts provided with pads, the attacks may be kept off for quite a 
considerable time. At the celebrated ball which every year at mi- 
careme is given to the hysterical and hystero-epileptic patients of 
the Salpetriere, in which, of course, only females take part, each 
dancer wears her belt. If this, owing to the movements in danc- 
ing, slips from its proper place, so that the pressure is taken off 
the ovaries even for a moment, a major attack comes on, and the 
patient, twisting and turning herself and presenting the most in- 
credible distortions, is removed from the ball-room, without causing 
the least interruption in the dancing. 

The outlook for complete recovery in major hysteria is not 
favorable. All attempts to cure the patients remain in many 
instances fruitless, as we may observe in the Salpetriere, where 



602 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

some patients, in spite of the best care and the most excellent 
treatment, remain for years without presenting any marked or 
lasting improvement either with regard to the violence or the 
frequency of the attacks. 

Sometimes, especially if the patients come early enough 
under the care of the physician, immediate removal from their 
homes into an institution does much good. The attacks become 
rarer and cease entirely after a few months. The treatment in 
these institutions consists in the " feeding system," which we 
have mentioned on page 566, as well as the ice-cold douches, 
to which we have also alluded above. 

The brilliant success of Charcot in the treatment of hystero- 
epilepsy is due to these three factors: (1) The removal from 
home, (2) the cold douches, and (3) the feeding system. With 
the removal of the ovaries, the use of static electricity and the 
magnet, the results have been shown to be much less favorable, 
and we may consider that these procedures, so far as the treat- 
ment of the major attacks goes, have in the main been dis- 
carded (cf. the references to metallotherapy). 

Reliable and correct as are the descriptions given by the 
Charcot school of the major attacks, which we may incidental- 
ly remark are very rarely seen in Germany, accurately as we 
can follow up the different phases or periods of the attack in 
many such patients, we still must be very careful in accepting 
the accounts of the influence of hypnotism upon hystero-epi- 
leptics and the conditions produced thereby. 

In the Salpetriere the patients were hypnotized by means 
of fixation of the eyes, by the action of a bright light, or the 
sound of an instrument called a tam-tam, or by similar means ; 
and, as every one must know who has been present at Charcot's 
experiments, certain individuals were hypnotized in a very few 
seconds. According to Richer, who, as we said, has made the 
most careful studies of this subject in conjunction with Char- 
cot, which appeared in various numbers of the Arch, de Neurol, 
from 1 88 1 to 1883, there may be distinguished four different 
stages: (1) The cataleptic, (2) the stage of suggestion, (3) the 
stage of lethargy, (4) the stage of somnambulism. 

In catalepsy, whether artificially produced or whether oc- 
curring spontaneously, as it does in hysteria in very exceptional 
cases, the members of the body remain in any position into 
which they have been put. Thus, if we passively bend the arm 
at the elbow and raise it up, it remains fixed in this position. 



HYPNOTISM. 



603 



Flexion or extension in any joint can be produced without the 
slightest resistance on the part of the patient — " flexibilitas 
cerea" ; even the most unusual, uncomfortable, and strangest 
attitudes are retained without any difficulty. How this most 
remarkable regulation of the necessary innervation is brought 
about we do not know as yet, neither have we the slightest 
grounds whereupon to base any theory by which we could 
seek to explain this condition, w 7 hich is not infrequently also 
associated with disturbances of consciousness. 

The state of hallucinations excited by slight stimulation of 
the special senses (in reality by suggestion), and designated as 
automatism, is characterized by total analgesia. The eyes re- 
main open, and it is a remarkable fact that positions which are 
given to the body evoke the corresponding expressions of the 
face, and, vice versa, the body assumes the corresponding posi- 
tion if on the face, by faradization of the muscles, a certain ex- 
pression — e. g., of sadness, hilarity, spite, voluptuousness, or 
fear — is produced. By firmly shutting the eyes of the patient 
it is claimed that the second stage may be converted into the 
third, the automatic into the stage of lethargy. In this latter 
the excitability of all the nerves and muscles is greatly in- 
creased, so that, for instance, slight pressure upon the stem of 
the facial nerve suffices to bring about contractions in all the 
muscles supplied by that nerve. The contraction lasts much 
longer than the stimulation, and therefore takes on a tetanic 
character. At the same time the patient is apparently com- 
pletely unconscious, and there is total anaesthesia. Now it is 
impossible to create hallucinations. The tendon reflexes are 
greatly exaggerated. If we now stroke the patient lightly over 
the top of the head, the hyperexcitability vanishes and a new 
stage comes on, that of the hysterical somnambulism. In this 
condition the patient is susceptible to external influences, inas- 
much as the organs of special sense are performing their func- 
tions to a certain degree. He answers questions (with closed 
eyes and, as it were, automatically), carries out instructions, 
and so forth. By local stimulation of the skin — for instance, 
by vigorous rubbing — we are able to produce contractures. 
By energetic pressure upon the eyes the patient can again be 
transferred from the somnambulistic to the lethargic condi- 
tion. The occurrence of hallucinations and illusions is not 
constant. 

For a long time the theory that this condition was peculiar 



604 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

to hysterical patients when hypnotized, just as the other condi- 
tions were peculiar to them when awake, was not doubted, and 
the so-called major hypnotism, as the hypnosis of the hystero- 
epileptics was called, created everywhere great astonishment 
and admiration, especially in those who could actually observe 
it in Charcot's clinic at the Salpetriere. It is only more re- 
cently that doubts have been raised about the correctness of 
these claims of Charcot. Many are inclined to believe that the 
above-described four stages, which the hypnosis of hysteria 
presents, can be produced in any hypnotized individual, and not 
only in those who are hysterical, and that therefore the " major 
hypnotism " is no neurosis at all and has no characteristics of 
its own. Whether the members of the Salpetriere school will 
be able to defend their former assertions, and what arguments 
they can put forth, and whether they will be able to continue 
to uphold the existence of different stages of hysterical hypno- 
sis after all possible sources of error have been excluded, we 
can not tell. How they will be able to demonstrate the neuro- 
muscular hyperexcitability as physiological and not perhaps as 
produced voluntarily, as many are inclined to think now, has to 
be left to the future to decide, and more especially to the abso- 
lutely necessary repetition of the experiments. Here it is our 
part only to show on what grounds Charcot's doctrines have 
been attacked, what proofs have been brought forward to show 
his doctrine to be untenable, and to state clearly the stand- 
point which is now generally held as regards the origin and 
the phenomena of hypnotism. 

This is not the place to enter into a consideration of the 
mysticisms and the charlatanisms of a man who a hundred 
years ago propounded the doctrine of the so-called magnetic 
fluid, which, emanating from the magnetizer, and being capable 
of spreading itself in space, could receive all impulses of mo- 
tion and impart them, but as a matter of historical interest and 
justice we are compelled to state that it was Franz Mesmer, 
born in 1733, who gave the first impulse to a movement which, 
founded on his arrogant and wild teachings, has passed through 
manifold phases, and to-day still exists, now that it has been 
found possible to sift the chaff from the wheat. Magnetism to- 
day has succumbed to the same fate as alchemy, and has been 
discarded, but both bore good fruit ; the one opened the door 
to chemistry, the other to hypnotic suggestion (Bernheim). 

The fact that there is no such thing as a magnetic fluid, that 






HYPNOTISM. 



605 



hypnosis and the phenomena occurring- during- it are entirely 
subjective in nature, and are to be attributed to external in- 
fluences upon the nervous system, was discovered by James 
Braid, of Manchester, in 1841, and we are justified in opposing 
" braidism " to " mesmerism " just as we oppose truth to false- 
hood. Braid concentrated the attention of those he wanted to 
put to sleep by making them keep their eyes fixed upon a 
bright object ; he assumed that the fatigue of the levator pal- 
pebral superioris, which was simultaneously produced, was the 
cause of a sleep during which the imagination was so active 
that spontaneous mental pictures, as well as impressions im- 
parted by others (" suggestions "), obtained the power of actual 
perceptions. If such impressions are imparted frequently, ac- 
cording to his observation, a certain habit is established, so that 
it becomes, cceteris paribus, easier and easier to put the patient 
to sleep. Braid was also acquainted with the fact that corre- 
sponding sensations and passions can be produced in hypno- 
tized persons by putting their facial muscles and their extremi- 
ties in appropriate positions, although he made no attempt to 
explain these phenomena physiologically. This has only been 
done quite recently, and even then the study was evoked only 
by a purely external stimulus, viz., the exhibitions which a 
Danish magnetizer named Hansen gave in the German cities. 
The impression which these made upon the public at large was 
of such an exciting and uncanny nature, and the whole thing 
was so puzzling to men of learning, that physiologists and neu- 
ropathologists were impelled to approach the subject to see 
whether the apparently supernatural and inexplicable could 
not be traced to natural physiological laws. One of the most 
prominent physiologists, Heidenhain, put forward the theory 
that, by weak but steady stimulation of the nerves of special 
sense, the cells of the cerebral cortex were induced to discon- 
tinue for a time their activity, thus causing the subcortical 
reflex centres to fall into a state of irritation, partly because, 
owing to this inactivity, the reflex inhibitory influence of the 
cortex was suspended, and partly because every impulse reach- 
ing the brain was propagated to a limited area which neces- 
sarily led to stronger excitation of the part of the excito- 
motor apparatus belonging to it. With this ingenious hypoth- 
esis, which many others — Weinhold, of Chemnitz, Griitzner, 
Rumpf, Berger, and Schneider among them — have accepted, 
we had to be satisfied, and for the physiologists the interest 



606 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

in the matter was thus exhausted, and the subject was aban- 
doned. 

In pathology and general practical medicine, including, as 
we shall see, surgery and obstetrics, the matter obtained a new 
and increased significance when, more recently, the observa- 
tions, which twenty years before had been made by an investi- 
gator in Nancy, Liebeault, were again taken up. Liebeault had 
published a work in 1866 with the title Du sommeil et des 
etats analogues consideres surtout au point du vue de Taction 
du moral sur le physique, in which he expanded the observa- 
tions of Braid ; he showed that it only needed a concentra- 
tion of the attention on a single idea, viz., the idea of going 
to sleep, to make the body immobile, and to produce a certain 
kind of sleep, which, however, differs from the physiological 
form (suggestion theory of hypnotism). The same author was 
the first to show that neither an optical, an auditory, nor a tac- 
tile stimulus was necessary to bring about hypnosis, but that 
the impressions from outside, the suggestions that the sleep 
must and will occur, are perfectly sufficient ; the hypnotized 
sleeper — whose ideation, in contradistinction to that of the 
ordinary sleeper, remains in contact with that of the hypnotizer 
— can be influenced by the latter in his ideas and actions. The 
fundamental observations of Liebeault remained unappreciated 
for twenty years ; the work was not read, hypnotism remained 
a curiosity, and it seemed inadvisable for a scientific physician 
to occupy himself with it, unless he were willing to gain for 
himself the reputation of a charlatan or of a man whose actions 
were suspicious or even dangerous. The credit of bringing 
to light the work of Liebeault, we might almost say of having 
discovered Liebeault, belongs to Bernheim, of Nancy, whose 
merit was still more augmented by his own contributions to 
the subject. He published his first article on hypnotism in 
1884, and with his book, De la suggestion et de ses applica- 
tions a la therapeutique, he has, to use a popular but expressive 
phrase, " hit the nail on the head." He and the Nancy school 
have to be regarded as the founders of the successful attempt 
to make a systematic use of hypnotism for therapeutic pur- 
poses, and should the treatment by suggestion ever be gen- 
erally accepted, and become an integral part of our thera- 
peutic armamentarium, although at present there seems little 
prospect of this, Bernheim will be mentioned as its scientific 
originator. For the adverse attitude which prominent clini- 



HYPNOTISM. 607 

cians and physicians in general show even to-day toward the 
treatment by suggestion there exist a variety of reasons which 
it is not necessary to discuss in the present work. This one 
point only need be emphasized here. In order to employ the 
treatment by suggestion with any real success, not only time 
and patience, but, above all, much experience is needed, which, 
of course, not every one possesses. Curiously enough, there 
exists, even among medical men, a widespread naive opinion 
that anybody can hypnotize, and that the treatment by sugges- 
tion is a branch of therapeutics that comes to a man without 
any study or practice. It is interesting and even, in a way, 
amusing to see how many, especially of the younger physi- 
cians, who have had a chance to observe the results of the 
treatment, make a few attempts at random, and if they do not 
succeed almost from the very first in obtaining good results, 
immediately begin to talk and write about the treatment as 
" humbug," which once for all should be regarded as unscien- 
tific. The habitual use of hypnotism is denounced as danger- 
ous, the condition produced as a pathological one which may 
ruin the whole organism, or at least the nervous system, etc. 
Nobody, certainly, who is acquainted with hypnotism will 
deny that pathological conditions may be produced by it and 
that it may be dangerous, but is this a ground upon which to 
simply discard it without a further hearing? Have we given 
up chloroform narcosis because it has now and then proved 
dangerous in the hands of the inexperienced and careless oper- 
ator, or have we given up the use of morphine on account of 
its poisonous action when used too freely and for too long a 
period of time ?• As in all other measures, we must recognize 
here indications and contraindications, and this can be done in 
the majority of cases without diffigulty ; and as everything in 
this world, especially in the practice of medicine, even the 
smallest operation — that of vaccination, for example — has to be 
learned, so the art of hypnotizing has to be acquired, and one 
can expect to comprehend the subject and to have success with 
the practice of the treatment by suggestion only after careful 
and painstaking study. 

It is very important to remember that it is never necessary 
to produce sleep in order to achieve therapeutic results, and 
the terms " hypnosis, hypnotizing, hypnotism," are therefore 
not well chosen. Only a moderate degree of bodily and men- 
tal fatigue suffices for the production of excellent results, and 



6o8 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

it is entirely unnecessary to bring about a hypnotic condition 
with amnesia, which, if repeated frequently, would undoubt- 
edly have a bad influence upon the patient. This mild degree 
of fatigue is produced as follows : The patient, having been 
placed in a comfortable armed chair, is asked to think of noth- 
ing else than of going to sleep. We " suggest " to him that he 
is beginning to feel tired, that he is no longer able to com- 
pletely open his eyes, which are already beginning to close, 
etc. At the same time he is asked to look steadily at two fin- 
gers of the hypnotizer, which at first are held directly in front 
of his eyes, but are gradually lowered, by which procedure the 
closing of the eyes, which we desire, is easily accomplished. 
Now either a difficulty in moving the arms or legs is suggested, 
a loss of sensation in certain parts of the skin, or some similar 
idea. The tone of voice in which all this is said should not be 
loud, but monotonous. The same suggestions must again and 
again be repeated, and care must be taken that disturbing 
noises, the slamming of doors or the striking of clocks, and 
such like, be not heard, so that the mind of the patient may as 
much as possible be concentrated upon the hypnotizer. Some- 
times, but by no means always, the very first attempt to bring 
about hypnosis is successful, as I have seen in some of Forel's 
as well as Wetterstrand's cases, and the hypnosis may be so 
profound that we can already venture to give therapeutic sug- 
gestions. Sometimes the first, second, and third attempts fail 
completely or partially ; then we must, if no contraindications 
exist, try again and again, but under no consideration should 
the individual trials be prolonged beyond two or three min- 
utes. Without question external circumstances are of great 
significance. If a patient who is to be hypnotized enters a 
room, in which eight, ten, or twelve persons are lying sound 
asleep stretched out on easy-chairs and sofas, and is left sitting 
there quietly for a quarter of an hour without any attempt to 
put him to sleep, his suggestibility — that is, his susceptibility — 
will sometimes be materially increased, and it will be a com- 
paratively easy matter to hypnotize him. But there are cer- 
tain internal conditions also which may throw great obstacles 
in our way, and which must, therefore, not be overlooked. 
Thus, if a patient does not believe that he can be put to sleep, 
or if he makes up his mind to resist us, a certain amount of 
finesse is necessary ; we have to outwit him in order to produce 
hypnosis without his consent or even against his will. Such 



HYPNOTISM. 



609 



exceptions, and the behavior of the physician who has to con- 
tend with them, can not here be treated of. Only one artifice 
we may mention which we have repeatedly used with very 
good results in producing the fatigue quickly and surely. We 
apply a large curved sponge electrode (anode) to the forehead, 
a second to the neck, close the circuit and allow a very weak 
(constant) current, just sufficient to produce the characteristic 
taste upon the tongue, to pass through the head for a few sec- 
onds, and then, without the knowledge of the patient, open 
the circuit and tell him that the electricity passing through the 
brain will put him to. sleep, and as a matter of fact this "sug- 
gested " current does so very promptly and surely. Secondly, 
the mental condition of the patient may stand in our way. It 
is an observation confirmed by all investigators that it is diffi- 
cult or impossible to hypnotize insane patients, and that hys- 
terical patients and hystero-epileptics are the least favorable 
subjects. In the domain of psychiatry the treatment by sug- 
gestion, so far as we can judge at present, remains without 
significance ; on the other hand, it seems as if certain disturb- 
ances in nutrition — for example, general anaemia and chlorosis 
— facilitate hypnotization greatly, while an absolute confidence 
in the physician, the absence of all attempts to analyze and to 
test our procedures on the part of the patient while we are 
trying to hypnotize him, will also materially increase the sus- 
ceptibility to suggestions. If all factors, favorable and unfa- 
vorable, are taken together, we may say that by far the greater 
number of people can be hypnotized ; perhaps one might go so 
far as to say all, without exception, are susceptible if time and 
circumstances allow sufficient repetitions of the trial. For hos- 
pital practice the dictum of Bernheim may for the present be 
accepted, that the physician who does not succeed in hypno- 
tizing eighty per cent of his patients for therapeutic purposes 
does not understand the method. 

The manner in which hypnosis comes on and the phenomena 
observed during this state are extremely varied. Sometimes 
the eyes close suddenly and the patient is asleep at once ; more 
frequently this is preceded by twitchings of the lids and moist- 
ure in the eyes, which are repeatedly closed and opened. 
Sometimes the lids are shut during hypnosis, sometimes a fine 
tremor is noticeable in them ; again, fibrillary twitchings in the 
muscles of the face may be remarked. The hypnotic influence 
does not always produce sleep, and, as we have said, this is not 
39 



6 io DISEASES OF THE GENERAL NERVOUS SYSTEM. 

necessary for therapeutic purposes ; but there are different de- 
grees, from the waking state to slight dullness of the senses and 
somnolence, and, finally, deep sleep, which latter is called som- 
nambulism. Bernheim in his explanation bases his arguments 
upon the ideas of Luys, that the different layers of the cortex 
are endowed with different functions : those nearest the surface 
are supposed to serve for the sensorium, the middle ones for 
the mental faculties, and the deepest for the transference of the 
will. He distinguishes accordingly nine degrees of hypnosis, 
and characterizes them in the following manner : (i) The pa- 
tient remains quiet with closed eyes during the suggestion, but 
can open them without difficulty when asked to do so, and 
claims not to have slept at all. (2) The patient is not able to 
open his eyes when asked. (3) The patient presents suggested 
catalepsy and analgesia, and remains in the position in which 
he is placed, but is able, after it has been suggested to him, to 
change from one position to another without assistance. (4) 
The patient is no longer able by himself to overcome the sug- 
gested catalepsy, and automatic, rotatory movements, especially 
of the arms, can be evoked. (5) Besides the catalepsy, con- 
tractures can be produced which the patient himself is not able 
to do away with. (6) The patient presents an automatic obe- 
dience ; he stands motionless if ordered to do so, he rises, 
walks, and acts, in fact, just as the hypnotizer may suggest. 
Intelligence and the activity of the senses are intact in these 
six stages. The patient on awakening remembers everything 
that has been done to him. (7) In the seventh stage the patient 
presents the same phenomena as in the preceding six stages, 
but on awakening has quite forgotten what has been going on. 
(8) Besides this amnesia on coming to, hallucinations can be 
produced during hypnosis which vanish after the return to the 
normal condition. (9) The suggested hallucinations persist 
after waking up — post-hypnotic suggestions — everything that 
can be produced in a patient when in a state of hypnosis can 
be brought about after he has awakened simply by suggesting 
to him during hypnosis that it will happen after he has awak- 
ened. In this possibility, of exerting an influence upon the pa- 
tient for a longer or shorter time after he is awake, lies the whole 
therapeutic significance of the treatment by suggestion. This 
(post-hypnotic) action, which in certain cases can be obtained 
in no other way than by suggestion, is sufficiently important 
to warrant and insure to hypnotism a lasting place in science. 



HYPNOTISM. 6ll 

It is unnecessary to distinguish nine different stages of hyp- 
notism as Bernheim did ; three are quite sufficient (Forel). 
The first is the stage of somnolence, corresponding to Bern- 
heim's first stage ; the second is that of hypotaxia (light 
sleep), embracing all the stages from the second to the sixth 
of Bernheim ; the third is the stage of deep sleep (somnam- 
bulism), corresponding to the seventh, eighth, and ninth of 
Bernheim's classification. It is of practical importance to 
note that frequent trials usually increase the susceptibility of 
the patient, and that as a result it is usually quite easy to 
produce the condition of fatigue (" somnolence ") necessary 
for therapeutic purposes. 

What are, then, the diseases in which we can, with good 
conscience and good hopes of success, venture to employ the 
treatment by suggestion ? We need hardly say that affections 
in which we have to deal with inflammatory processes, new 
growths, infections, or, in a word, with organic lesions, do not 
belong to this class ; and, as a matter of fact, it would hardly 
enter any one's head to attempt to cure pneumonia, typhoid 
fever, brain tumors, syphilis, tetanus, etc., by means of hypno- 
tism. It is a different matter if we are dealing merely with cer- 
tain symptoms of such maladies — for instance, insomnia, diffi- 
culty in breathing, or pains of the most varied kinds, not exclud- 
ing the lancinating pains of tabes. Here hypnotism should, at 
least, be given a trial, yet the main field in which the treatment 
by suggestion should be employed will not be the diseases we 
have mentioned, but rather all those which we have designated 
and described as functional disorders of the nervous system. 
Here motor as well as sensory disturbances can be influenced, 
the latter having, cceteris paribus, a better prognosis. Neural- 
gias, especially tic douloureux, are often difficult to treat, and 
the migraine-like paroxysmal headaches can not always be per- 
manently removed. Among the general diseases of the nerv- 
ous system, epilepsy, the classical, hereditary migraine, and 
hysteria, as a whole, have a very unfavorable prognosis. On 
the other hand, certain individual symptoms of hysteria (the 
vagus-neuroses, anaesthesias, paralyses) are very amenable to 
the treatment. Further details relating to this subject I have 
treated of in a paper read before the International Congress in 
Rome (Wien med. Presse, 1894, 22), to which the reader is re- 
ferred. I would call attention again, however, to the treatment 
of alcoholism and of certain functional speech disturbances 



612 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

(stuttering, stammering), since my results in these conditions 
were especially favorable. 

According to Forel, it is possible to influence certain so- 
matic functions to some extent — e. g., the menstruation and 
digestion — in such a manner that the menses can be brought on 
at a certain day and a certain hour, and a regular evacuation 
of the bowels every day can be insured by suggestion. Al- 
though these accounts come from the most indubitable source, 
the experiments must again be tested and confirmed. They 
can certainly only be successful, we should think, in individuals 
who have been repeatedly hypnotized and are, as it were, 
"trained." With the treatment of alcoholism by suggestion 
Forel also has had uncommonly good results in his insti- 
tution. The heaviest drinkers were not only for a time, but 
lastingly cured ; but no little influence certainly has here to be 
attributed to the temperance societies of which such individ- 
uals were led to become members. I have been able to obtain 
good results without this help. The behavior of morphinists 
toward suggestion requires further study. The results so far 
obtained seem not to be very encouraging. The communica- 
tion of Wetterstrand (cf. lit.) that it is possible in idiopathic 
epilepsy to diminish the frequency and severity of the attacks 
deserves to be remembered, and the procedure should be tried 
in cases in which bromides are not well borne. Finally, we 
would call attention to the anaesthesia and analgesia which can 
easily be produced by suggestion, and which in surgery, as 
well as in obstetrics, may be very useful. I was present at 
Forel's clinic at the extraction of two obstinate teeth, which, 
after the proper hypnotization, were taken out without the 
slightest sign of pain on the part of the patient. Possibly the 
pains during labor may be removed by hypnotism. The an- 
aesthesia of the mucous membrane of the fauces may be very 
valuable in making laryngoscopical examinations and the like. 



HYPNOTISM. 613 

LITERATURE. 

I. Hypnotism — Treatment by Suggestion (from the Year 1887). 

a. General. 

All the older references have been collected by Mobius (cf. Schmidt's Jahrbucher, iS8i, Bd. 

190. P- 73)- 

Berillon. La suggestion et ses applications a la pedagogic Gaz. des hop., 

1887, 123. 

Binswanger. Deutsche med. Wochenschr., 1887, xiii, 42. (Present State of 

Hypnotism.) 
Fontan et Segard. Elements de medecine suggestive. Paris, O. Doin, 1887. 
Bernheim. De la suggestion et de ses applications a la therapeutique. Paris, 

1888, deuxieme edition. 

Baierlacher. Munch, med. Wochenschr., 1888, xxxv, 30. 

Krafft-Ebing. Eine experimentelle Studie auf dem Gebiete des Hypnotismus. 
Stuttgart, 1888.— English translation by Charles G. Chaddock. G. P. Put- 
nam's Sons. The Knickerbocker Press, 1889. 

Maack. Zur Einfuhrung in das Studium des Hypnotismus und thierischen Mag- 
netismus. Berlin und Neuvvied, 1888. 

Schrenck-Notzing, v. Ein Beitrag zur therapeutischen Verwerthung des Hyp- 
notismus. Leipzig, Vogel, 1888. 

Seeligmuller. Der moderne Hypnotismus. Deutsche med. Wochenschr., 1888, 
xiv, 31-34. 

Sallis. Ueber hypnot. Suggestionen, deren Wesen. klinische und strafrechtliche 
Bedeutung. Neuwied, 1888. 

Corey. Boston Med. and Surg. Journ., November 20, 1888, lxix. (Therapeutic 
Value of Hypnotism.) 

Forel. Schweiz. Corresp.-BL, 1888, xviii, 23. (The Value of Hypnotism for the 
General Practitioner.) 

Herter. Boston Med. and Surg. Journ., November 20, 1888, cxix. 

Mason. Ibid., November, 1888. 

Bernheim. Hypnose durch Suggestion. Wien. med. Presse, 1888, xxviii, 26. 

Jendrassik. Neurol. Centralbl., 1888, 10, n, 

Meynert. Ueber Hypnotismus. Wien. med. Presse, 1888, xxix, 24. 

Weiss, D. Prager med. Wochenschr., 1888, xiii, 20, 21. 

Freud. Wiener med. Blatter, 1888, xi, 38, 39. 

v. Krafft-Ebing. Ueber Hypnotismus. Deutsche Med.-Ztg., 1888, 16, p. 196. 

Dessoir. Bibliographie des modernen Hypnotismus. Berlin, 1888. 

Liebeault. Du sommeil provoque. Deuxieme edition, Paris, 1889. 

Liegeois. De la suggestion et du somnambulisme dans leurs rapports avec la 
jurisprudence et la medecine legale. Paris, Doin, 1889. 

Baierlacher. Die Suggestiv therapie und ihre Technik. Stuttgart, 1889. 

Beaunis. Le somnambulisme provoque. Etudes physiologiques et psycholo- 
giques. I2e, Paris, 1886. 

Binswanger. Therap. Monatsh., 1889, 1-4. 

Gilles de la Tourette. Der Hypnotismus vom Standpunkte der gerichtl. Medicin. 
Author. German translation, Hamburg, 1889. 



6 14 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Moll. Der Hypnotismus. 2. Auflage, Berlin, 1890. 

Pitres. De la memoire dans 1'Hypnotisme. Gaz. med. de Paris, 1890, No. 47. 

Forel. Der Hypnotismus, seine psycho-physiol. u. s. w. Bedeutung und seine 

Handhabung, 2. Aufl., Stuttgart, 1891. 
Wetterstrand. Der Hypnotismus und seine Anwendung in der praktischen 

Medicin, Wien und Leipzig, 1891. 
Moll. Der Rapport in der Hypnose. Untersuchungen iiber den thierischen 

Magnetismus. Leipzig, 1892. 
Liebeault. Der kiinstliche. Schaf und die ihm ahnlichen Zustande. German 

translation by Dornbliith. Leipzig und Wien, Deuticke, 1892. 
Binswanger. Ueber die Erfolge der Suggestivtherapie. Wiesbaden, 1892. 
Grossman. Zeitschrift fur Hypnotismus, Suggestionstherapie, u. s. w., 1893, iii, 

Jahrg., Berlin, Herm. Brieger. 
Hirsch. Suggestion und Hypnose. Kurzes Lehrbuch fiir Aerzte, Leipzig, Abel, 

1893. 
Hecker. Ueber Autosuggestionen wahrend des hypnotischen Schlafes. Zeitschr. 

f. Hypnot., ii, 1, 17. 
Kuhner. Psychotherapie. Der prakt. Arzt, 1893, 5. 
v. Corval. Suggestionstherapie, Psychotherapie. Eulenburg's Real-Encyclo- 

padie, 2. Aufl. 
Benedict. Hypnotismus und Suggestion, Eine klinisch-psychologische Studie. 

Leipzig und Wien, 1894. 
Grossmann. Die Bedeutung der hypnotischen Suggestion als HeilmitteL 

Gutachten und Heilberichte der hervorragendsten wissenschaftlichen Ver- 

treter des Hypnotismus der Gegenwart, Deutsche Ausgabe, Berlin, 1894. 

b. Special ( Various Cases cured or treated by Hypnotic Suggestion). 

Sollier. Progres med., 1887,42. (Hystero-epileptic Attacks said to have been 

cured.) 
Mialet. Gaz. des hop., 1887, 116. (Hyperemesis Gravidarum cured.) 
Birdsall. Boston Med. and Surg. Journ., November 20, 1888, cxix. (Tremor.) 
Frey. Wien. med. Presse, cxix, 50, 51. (Neuralgia of the Fifth cured.) 
Frey. Ibid., xxix, 25. (Sleeplessness cured.) 
Baierlacher. Munchener med. Wochenschr., 1888, xxxv, 39. (Report of 

Cases.) 
Hackel. Die Rolle der Suggestion bei gewissen Erscheinungen der Hysterie 

und des Hypnotismus. Jena, 1888. 
Forel. Schweiz. Correspond. -BL, 1888, xviii, 6. 
Nonne. Neurol. Centralbl., 1888, vii, 7, 8. 

Ribot. Revue med. de la Suisse rom., Mars, 1888, viii, 3. (Hysterical Hemi- 
plegia cured.) 
Scheinmann. Deutsche med. Wochenschr., 1889, 21. (Hysterical Aphonia 

cured.) 
Michael. Deutsche Med.-Ztg., 1889, 63. (Epilepsy Temporarily improved, 

Hystero-epilepsy and Hysterical Aphonia cured.) 
Barth. Suggestion bei Ohrenleiden. Zeitschr. f. Ohrenhk., 1889, xix, 3, 

p. 231. 
Ladame. Internat. klin. Rundschau, 1890, 21, 22. (Craving for Alcohol 

cured.) 



HYPNOTISM. 



615 



v. Schrenck-Notzing. Die Suggestionstherapie bei krankhaften Erscheinungen 

des Geschlechtssinns. Stuttgart, 1892. 
Donath. Deutsche Zeitschr. f. Nervenhk., 1892, 2 und 3. 
Stembo. Die therapeutische Anwendung der prahypnotischen Suggestion. 

Petersburger med. Wochenschr., 1892, 37. 
Hitzig. Schlafattaken und hypnot. Suggestion. Berliner klin. Wochenschr., 

1892, 38. 
Grossmann. Die Erfolge der Suggestionstherapie bei Influenza. Berlin, 

Brieger, 1892. 
Schaffer. Netzhautreflexe wahrend der Hypnose. Neurol. Centralbl., 1893, xii, 

23, 24. 
Tatzel. Drei Falle von nicht hysterischen Lahmungen und deren Heilung 

mittelst Suggestion. Zeitschr. f. Hypnot., 1893, ii, 1. 
Forel. Die Heilung der Stuhlverstopfung durch Suggestion. Eine praktische 

und theoretische Studie. Berlin, 1894. 



PART II. 

DISEASES OF THE GENERAL NERVOUS SYSTEM 
WITH KNOWN ANATOMICAL BASIS. 

The anatomical changes, which are found in the diseases 
belonging to this category, concern the central nervous system 
as well as the peripheral nerves. The former always suffers, 
the latter are only in certain cases affected. Whether the 
changes in the peripheral nerves are to be regarded as second- 
ary, or whether the entire nervous system becomes affected in 
all its parts at the same time, so that the peripheral and the 
central lesions progress pari passu, can not be definitely de- 
cided. The nature of the anatomical changes will be discussed 
under the head of each individual affection. Combinations of 
the functional neuroses and organic diseases of the nervous 
system are, on the whole, rare. Such instances have been care- 
fully studied by Oppenheim (Neurol. Centralblatt, 1890, 16). 



CHAPTER I. 

MULTIPLE SCLEROSIS — DISSEMINATED SCLEROSIS INSULAR SCLE- 
ROSIS — SCLEROSE EN PLAQUES SCLEROSIS CEREBRO- 

SPINALIS DISSEMINATA S. MULTIPLEX. 

Although multiple sclerosis is not one of the common 
affections of the nervous system, it is desirable and important 
for the general practitioner to possess a clear understanding of 
it, because the clinical appearances by which the different cases 
manifest themselves vary within such wide limits and remind 
us now of this, now of that spinal or cerebral affection, with- 
out ever completely simulating any one definite disease. The 
typical course given in the books is not very often met with 
in practice. Much more commonly one or the other of the 
classical symptoms is not found at all, or, if present, is only very 
slightly developed. On the other hand, symptoms are occa- 
616 



MULTIPLE SCLEROSIS. 



6. 7 



sionally encountered which are not included in the usual de- 
scriptions of the disease. In a word, multiple sclerosis is quite 
inconstant in its manifestations, a circumstance which often 
makes the diagnosis very difficult. In the investigation into 
the pathology as well as the clinical aspect, Charcot has done 
admirable and lasting service. 

Symptoms and Course. — The course of a classical case is 
usually as follows : The patient first complains of general symp- 
toms — headache, vertigo, digestive disorders — soon, also, of 
sensory disturbances in the upper and lower extremities, slight 
weakness, and a readiness to become fatigued. These symp- 
toms may persist for months, yet relatively early one or several 
apoplectiform attacks may occur which sufficiently indicate 
the seriousness of the condition. It strikes the patient, as well 
as those who surround him, as a peculiar thing, that whenever 




ii-n^siAL 






*r* 



VM. 



Fig. 164. — Specimen of Handwriting in a Case of Multiple Sclerosis. (Ich heise 
(heisse) Henriette Sterner, bin 48 Iahre alt. Breslau, den 19 Juni.) 

he attempts to pick up something with his hands, or to make 
any other movement, a tremor appears, in exceptional cases 
implicating the facial muscles also (Cohn, Deutsche med. Wo- 
chenschr., 1890, 13), but usually confined to the upper extremi- 
ties, which frustrates the intended movement more or less com- 
pletely. If he attempts to raise a full glass to his mouth, he 
spills some of the contents. If he attempts to eat, the food is 
jerked off his fork, etc. Co-ordinated movements, such as are 
required for writing or playing the piano, become difficult, the 
handwriting becomes almost illegible (Fig. 164), and the condi- 



6i8 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

tion is materially aggravated if the tremor is not confined to 
the upper, but if also the lower extremities, the trunk, neck, 
and head are attacked, so that on voluntary movements — on 
attempts to walk, for instance — the whole body first begins to 
tremble, and finally shakes so violently that the patient is forced 
to sit or lie down at once. This symptom, which is almost 
pathognomonic for multiple sclerosis, or at any rate most sig- 
nificant, is called "intention tremor," a term which does not, 
however, imply that the tremor is " intentional," but only that 
it appears on voluntary (" intended ") movements. During rest 
no trace of it is observed. When the patient lies quietly and 
undisturbed in bed no tremor is present, whereas, if he is 
spoken to, examined, made to answer questions, and the like, a 
tremor over the whole body develops, which, of course, pre- 
sents various degrees of intensity. It is most marked and 
characteristic if the patient is asked to bring his hand slowly 
to an object — for instance, to a pin laid upon the table. At first 
the motion is fairly good and steady, he trembles but little or 
not at all, but the closer he approaches to the pin the more un- 
steady becomes the hand and the larger become the excur- 
sions of the tremor, so that to grasp the pin becomes impos- 
sible. In some exceptional cases I have seen the shaking 
movements appear on one side only, so that the patient was 
capable of performing normal movements with one hand and 
one leg, when those of the other side had become entirely 
useless. 

In this intention tremor the eye muscles also take part; 
as soon as the patient attempts to fix a point with his eyes 
nystagmus appears, which, however, differs from the tremor 
of the other voluntary muscles, inasmuch as it does not com- 
pletely disappear during rest. As a subjective symptom the 
very annoying sensation of giddiness must be mentioned in 
this connection, which leaves the patient only when he lies 
quietly in bed, whereas it otherwise impedes him a good deal 
in his movements, especially in walking. Owing to the faulty 
innervation of the tongue and larynx, we meet with a peculiar 
speech disturbance ; the patient talks slowly, in a monotonous 
tone, and awkwardly, and his speech is scanning, as he makes 
a pause after each word, almost after each syllable, so that it 
takes him a much longer time to express his thoughts than a 
healthy man: "Yes — doctor — I — am — very — much — fa — tigued 
— and — worn — out." As this is spoken in the manner indicated, 



MULTIPLE SCLEROSIS. 619 

without any change of intonation, it is very characteristic in- 
deed, and it is, together with the intention tremor and the 
nystagmus, pathognomonic for multiple sclerosis. It impresses 
itself so much upon the mind that once heard it can never be 
forgotten or misinterpreted. 

To give a physiological explanation of the intention tremor 
is out of our power, and it is more especially not clear why 
it is so extremely common in multiple sclerosis, where we have 
such an irregular distribution of the anatomical lesions, where- 
as in most of the other cerebral affections it is absent. Whether 
Charcot's idea is correct, according to which the long persist- 
ence of the axis cylinders in the sclerotic foci has some connec- 
tion with the tremor, or whether we should hold with Strum- 
pell that the loss of the myeline sheaths, in consequence of 
which an abnormal diffusion of the nerve current from fibre to 
fibre occurs, is responsible for this, we can not decide, nor have 
we any proof of the correctness of Stephan's view (cf. lit.) that 
the existence of sclerotic foci in the thalamus gives rise to the 
phenomena, nor of Cramer's (cf. lit.) that the intention tremor 
has to be explained as analogous to the tremor which comes 
on after hard muscular exertion. 

Though we may be justified in looking upon these three 
symptoms as constituting in a manner the typical picture of 
multiple sclerosis, we must, as we have said above, at once 
familiarize ourselves with the fact that even these may not all 
be pronounced, or, again, that there may be others to be found 
in conjunction with them, developing in the course of the dis- 
ease. Among these latter we may mention certain spastic 
symptoms — rigidity of the muscles, increased tendon and skin 
reflexes, the above-described spastic walk — which, together, are 
liable to simulate, at least for a time, the picture of spastic spinal 
paralysis. This is the more likely as there are no sensory dis- 
turbances at all to be noted in multiple sclerosis; only in rare 
exceptions paraesthesias are observed, owing to which tabes 
and myelitis may be diagnosticated, especially if, as sometimes 
happens, bladder disorders are superadded. A careful study 
of the sensory changes has been made by Freund (Arch. f. 
Psych., 1890-92, p. 319). That bladder diseases are by no 
means so rare in multiple sclerosis as was formerly supposed, 
has been pointed out by Erb, and after him by Oppenheim 
(Deutsche Med.-Ztg., 1889, 32). Glycosuria will be found asso- 
ciated with the disease if there are foci situated in the floor 






620 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

of the fourth ventricle (Richardiere, Revue de med., Juillet, 
1 88;). 

Participation of the optic and other cranial nerves is not 
very rare, yet it is here much less important for the diagnosis, 
and much less significant for the course of the disease than, for 
example, in tabes. Diplopia is rarely met with; and equally 
uncommon is the neuritis and atrophy of the optic which leads 
to amaurosis. Uhthoff (cf. lit.) has pointed out, in an admirable 
study, that if optic atrophy occurs it is not like the primary 
atrophy in tabes, but that here it is a secondary process, which 
follows an active increase of the fine connective-tissue elements. 
It is self-evident that various disorders of sight are associated 
with this, yet they often present temporary improvement, and 
have usually a less serious issue than those of tabes. In gen- 
eral, it is characteristic of multiple sclerosis that its course is 
not uninterruptedly progressive, but that it shows remissions, 
during which the hopes of the patient as well as of his friends 
for his complete recovery are aroused. I have seen instances 
in which such remissions lasted for years and the symptoms 
disappeared to a great extent, and in which, just owing to this 
peculiarity in the course of the disease, the diagnosis could be 
made with some certainty. 

Cerebral manifestations are not uncommon, and frequently 
a slight degree of dementia develops, which to the patient 
himself makes his condition more bearable. It must also be 
regarded as a sign of beginning mental weakness, I think, that 
in some cases the patient frequently laughs boisterously without 
a cause. One of my patients had spells of loud laughter, which 
lasted from one to three minutes, and which appeared usually 
without sufficient motive. I have never had occasion to observe 
pronounced states of depression or exaltation in the course of 
this disease. The vertigo, w T hich of course must also be re- 
garded as a cerebral symptom, has been spoken of above. 
Apoplectiform attacks in the beginning of the disease are not 
rare ; epileptiform seizures may be found, if the cerebral cortex 
is more especially implicated. 

It has been shown by Charcot that in certain cases the 
development of the symptoms appears to be abortive and the 
affection, one might almost say, remains latent and can only be 
recognized by the peculiar shaking tremor. He proposed for 
these instances Trousseau's designation, "formes frustes" and 
it seems that in multiple sclerosis such forms are observed rela- 



MULTIPLE SCLEROSIS. 62 1 

tively frequently. Soucques studied these carefully under the 
direction of Charcot (Progres med., 1891, 1 1). As an example of 
the general course which the disease may run I insert here the 
following history of a patient in my wards, who is still living : 

Paul W., thirty-one years old, began to be sick ten years ago 
during his military service. At first, at times he could not feel his 
rifle in his left arm, and then in the same year he was often conscious 
of a slight feeling of fatigue, which was associated with vertigo. He 
had a good deal of difficulty with his arms and his legs ; they always 
felt as if they were asleep, and any muscular action necessitated 
the greatest exertion. He could not go through his salutes in the 
proper manner, and he was repeatedly punished on this account. 
At the same time he had now and then vomiting and weakness of 
the bladder for quite a long time, so that, on coughing, small quan- 
tities of urine were passed involuntarily. On examination, we are 
told, Romberg's sign was absent and the patellar reflexes were in- 
creased. A few months later, marked weakness in the right arm and 
the right leg became manifest, and the acuteness of hearing became 
diminished on that side. The patient complained of an annoying 
double vision. In 1879 he had some difficulty in swallowing; the 
bolus would stick in his throat, so that he had to force it down. In 
1880 pronounced deliberation in speaking is said to have been notice- 
able, and the patient at that time also complained that he could not lay 
his tongue upon certain words which he wished to use. The speech 
disturbance soon passed off, but the patient suffered from various 
troubles till January, 1884, in which month I saw him for the first 
time. He then presented the symptoms of an incipient tabes, but it 
was noted as a remarkable feature that the patellar reflexes were 
retained. The lancinating pains, however, the paresis of the legs, 
the diplopia, the paresis of the bladder, the unsteadiness which ap- 
peared especially in the dark, seemed sufficient to warrant the diag- 
nosis of tabes, and in the out-patient department this diagnosis was 
made, although with some reservation. The patient declined to 
enter the hospital. He was therefore ordered galvanism, but was 
lost sight of in the summer of 1884. Two years later he was treated 
at a hospital in this city for six months. Although I was unable to 
obtain a record of the case, I heard that the tabetic symptoms were 
very indistinct, and that the condition suggested rather a spastic 
paralysis. The patient was again lost sight of. Finally, on Janu- 
ary 8, 1888, he was admitted to the medical ward of the city alms- 
house, where he still remains. From a note made on January 10, 
1888, the following is extracted: The patient is a well-nourished 
man ; as he lies quietly in bed, the general aspect suggests nothing 



622 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



abnormal ; if, however, he is asked to perform any movement, the 
whole body — trunk, head, and extremities — is seized with a violent 
shaking tremor, which makes it difficult for him to get up, and 
impossible for him to walk without assistance from another person, 







even when supported by two canes. If he is allowed to discontinue 
all attempts at moving, the tremor gradually abates, and five or ten 
minutes later he is perfectly quiet again. The patient is unable to 
feed himself, and can not occupy himself with anything. The mus- 
cular strength is retained everywhere. In the domain of the cranial 



MULTIPLE SCLEROSIS. 



623 



nerves nothing but nystagmus can be noticed, which is especially 
well marked on the right side. The facial, hypoglossal, etc., are 
normal. The tongue is protruded steadily and straight. Speech is 
slow, although not distinctly scanning. There are no motor or sen- 
sory speech disturbances. The tendon reflexes in the upper, but 




more especially in the lower extremities, are increased, and the skin 
reflexes are without exception well marked. Sensory changes can 
nowhere be demonstrated, and the bladder disturbances, which were 
present on previous occasions, have disappeared. The spinal column 
is nowhere tender on pressure. Among the subjective complaints 



624 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

of the patient the dizziness is alone to be mentioned, which, however, 
even if the shaking movements were not present, in itself would be 
sufficient to keep him from doing anything. 

As a result of this examination the diagnosis of multiple sclerosis 
was made, and will certainly be proved to be correct at the post- 
mortem examination. It is interesting, however, that in this case 
the course of the disease suggested in its initial stages Thomsen's 
disease (although not congenital), later tabes (with retained knee- 
kick), then spastic spinal paralysis (conjectural diagnosis), before 
the picture of insular sclerosis developed. 

Diagnosis. — The diagnosis presents difficulties in almost 
every case, owing to the protracted course and the changes in 
the picture of the disease during the different periods. Even 
the most careful examination will not always keep us from 




MJ 



m, 



l/^C 



/m 



Fig. 167.— Specimen of Handwriting of a Patient (hat-maker) with a Mercu- 
rial Tremor. 

errors, and we must never be surprised if the autopsy does not 
always confirm the diagnosis made during life. The case of 
Westphal, in which a multiple sclerosis was diagnosticated, 
but where post mortem no lesions at all were found, has been 
alluded to before. In another instance, reported by Frey (cf. 
lit.), there was found, instead of the confidently expected foci 
of sclerosis, a leptomeningitis, and similar errors would not be 
difficult to find on a careful perusal of the literature. The pos- 
sibility that we are dealing with hysteria, in a given case, must 
always be considered, and then, of course, great weight must 
be laid upon the presence of other symptoms which would 
indicate such a condition. The difference between intention 
tremor, as illustrated in Fig. 164, and other tremors, can be 
seen by a comparison with Figs. 165-168. 



MULTIPLE SCLEROSIS. 



625 



Pathological Anatomy. — The anatomical changes of mul- 
tiple sclerosis are extremely characteristic. Even with the 
naked eye, here and there, grayish-white foci are seen in the 
brain, in the white matter of the hemispheres, in the walls of 




9 a 



II 

< Q 



in ^ 
H ° 

o 
o y 



c a 



I 



the lateral ventricles, in the corpus callosum, in the pons, and on 
its surface, in the medulla oblongata, in the floor of the fourth 
ventricle, and in the spinal cord, where the white matter is 
decidedly more affected than the gray. The foci are distrib- 
uted in a very irregular manner; sometimes they are more 

numerous in the brain, sometimes they are more numerous in 
40 



626 



DISEASES OE 7'HE GENERAL NERVOUS SYSTEM. 



the cord, often they are found scattered equally over the en- 
tire central nervous system. If they are situated on the sur- 
face, they are seen through the pia, and are somewhat more 
prominent than the parts which surround them. They are 
generally harder and firmer than the rest of the substance, and 
on section they assume a light-pink color when exposed to the 
air. If they are examined microscopically, they are found to 
consist of reticulated fibrillary supporting tissue, and contain 
only a few intact nerve fibres ; after the death of the medullary 
sheaths, the axis cylinders are preserved for an extraordinary 
length of time (Charcot). Secondary degenerations in the 
spinal cord are often absent (Strumpell), yet they are occasion- 
ally seen (Werdnig). The vessels show an increase in the 
nuclei, later a thickening of their walls, and are seen as yellow 
dots in the sclerotic foci. Whether the disease of the vessels 




Fig. 169.— Cross-section through the Cervical Enlargement of the Spinal 
Cord in a Case of Multiple Sclerosis. Hardened in osmic acid. The lighter 
colored areas in the white matter represent the sclerotic foci. (After Bramwell.) 



actually gives rise to the foci is not yet established (Fig, 169). 
Disease of the peripheral nerves has never been demonstrated 
in multiple sclerosis. 

Etiology. — About the aetiology we know practically noth- 
ing. It is possible that heredity deserves some consideration 
in this connection, but there are relatively many cases in which 



MULTIPLE SCLEROSIS. 627 

this factor can positively be excluded. The influence of infec- 
tious diseases upon the development of insular sclerosis has 
recently again been dwelt upon by Marie (cf. lit.). Typhoid 
fever, variola (Sottas, Gaz. des hop., 1892, 44), scarlet fever, 
measles, whooping-cough, influenza (Massalongo, Silvestri, 
Revue neurol., 1893, i, 23), and intermittent fever have repeat- 
edly been known to precede the disease, although the material 
at our disposal is not as yet sufficient to prove a causative rela- 
tion between the two. With regard to syphilis the connection 
here is by far less definite than, for instance, in tabes (cf. the 
case of Buss, lit.). Age and sex seem to be of some signifi- 
cance, inasmuch as children and aged people seem to be ex- 
empt. Westphal and others have only exceptionally seen it in 
children. Stnimpell has observed it in a man of sixty. Both 
sexes seem to be attacked with about the same frequency. I 
have seen a case in which after a severe traumatism (fall from 
a ladder) the three cardinal symptoms of multiple sclerosis de- 
veloped ; nevertheless I am not convinced that the case was 
not one of traumatic neurosis. The question can only be settled 
by the autopsy. 

Treatment. — An effectual treatment for multiple sclerosis 
does not exist. We possess no remedy which will arrest the 
development of the foci. The symptomatic treatment must 
always be tried, however, and the patient particular^ seeks 
relief from the annoying tremor. For this we may administer, 
although without raising our expectations too high, veratrine, 
physostigmine, one to three milligrammes (grs. ^-/ao) daily, in 
pills, or solanin, recommended by Grosset and Sarda, and even 
termed by these authors u medicament dn faisceau pyramidal" 
(Progr. med. 1888, 27). It may be given in doses of from two 
to three centigrammes (grs. %-/ 2 ) from three to five times 
daily. In other respects the treatment is the same as in mye- 
litis (cf. page 455). 

LITERATURE. 

Rovighi (Modena). Sclerosi multipla del midollo spinale complicata a Micosi. 
Reggio Emilia, 1884. 

Marie, P. Progres med., 1884, xii, 15, 16, 18. * (Multiple Sclerosis and Infec- 
tious Diseases.) 

Eulenburg. Neurol. Centralbl., 1884, 22. 

Hirt. Differentialdiagnose zwischen Hysterie und multipler Sclerose. Bresl. 
arztl. Zeitschr., 1885, vii, 11. 

Uhthoff. Ueber Neuritis optica bei multipler Sclerose. Berliner klin. Wochen- 
schr., 1885, 16. 



628 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Koeppen. Arch. f. Psych, u. Nervenkrankheiten, 1886, xvii, 1, p. 63. (Ana- 
tomical Examination.) 

Cramer, E. Ueber das Wesen des Zitterns. Aus der Krankenabtheilung des 
Breslauer Armenhauses. (Prof. Hirt.) Inaug.-Dissert., Breslau, 1886. 

Moncorvo. Sulla etiologia della sclerosi a placche mi bambini e specialmento 
sulla influenza patogenica della sifilida ereditaria. Napoli, 1887. 

Gilbert et Lion. Contribution a l'etude de la sclerose en plaques a forme para- 
lytique de la variete hemiplegique. Arch, de phys. norm, et pathol., 3me 
ser., Juillet, 1887, p. 126. 

Unger. Ueber multiple, inselfdrm. Sclerose im Kindesalter. Leipzig u. Wien, 
1887, Toplitz u. Deuticke. (Neurol. Centralbl., 1888, 2.) 

Oppenheim. Berliner klin. Wochenschr., 1887, 48. 

Stephan. Zur Genese des Intentionstremor. Arch. f. Psych, u. Nervenkrank- 
heiten, 1886, xviii, 3 und 1887, xix, 1. (With sixty-one references.) 

Buss. Berliner klin. Wochenschr., 1887, xxiv, 49. (Multiple Sclerosis in a 
Child with Hereditary Syphilis.) 

Werdnig. Ein Fall von disseminirter Sclerose des Riickenmarkes, verbunden 
mit secundaren Degenerationen. Med. Jahrb., Wien, 1889, Jahrg., 84. 
Heft 7, p. 335. 

Uhthoff. Untersuchungen iiber Augenstdrungen bei multipler Herdsclerose. 
^ Arch. f. Psych, u. Nervenkrankheiten, 1889, xxi, 1. 

Charcot. Sclerose en plaques et hysteric Gaz. hebd., 1889, 2me ser., xxvi, 7. 

Buss. Deutsches Arch. f. klin. Med., 1889, 5, 6. 

Chaslin. Arch, de med. experim., 1891, 3. 

Nolda. Arch. f. Psych., 1891, 2, p, 565. 

Glaser. Zeitschr. f. klin. Med., 1892, 3, 4. 



CHAPTER II. 

TABES DORSALIS — LOCOMOTOR ATAXIA (POSTERIOR SPINAL 
SCLEROSIS LEUCOMYELITIS POSTERIOR CHRONICA). 

The second of the diseases belonging- to this group cer- 
tainly deserves to be considered as one of the most important 
of those with which we are acquainted, not only because it is 
to be reckoned among the diseases of the nervous system which 
occur relatively frequently, and with which the general practi- 
tioner is not rarely brought face to face, but also because its 
clinical picture presents so many essential differences that it 
requires a large experience to feel at home with it on all occa- 
sions. Nobody questions the importance of the recognition of 
the disease in its early stages if only on account of its bearing 
upon the treatment, but many do not appreciate the difficulties 
which this early diagnosis entails. The more cases of tabes 
we see, the more we are surprised at the protean character of 
the symptoms, and the more are we convinced that almost 
every case offers some point of particular interest, and that 
occasionally even an expert can be sure of the diagnosis only 
after repeated examinations and long observation. 

LITERATURE. 
I. General, Monographs, etc. 

Adamkiewicz. Die Riickenmarksschwindsucht. Wien, Toplitz u. Deuticke, 

1888. 
Striimpell. Ueber Wesen und Behandlung der Tabes. Munch, med. Wo- 

chenschr., 1890, 39. 
Rouffilange. Contribution a l'etude du tabes et de l'hysterie. These de Paris, 

1893. 
Minor. Neurol. Centralbl., 1893. 
Mobius. Schmidt's Jahrbucher, 1894, ccxli, p. 73. (Ninth Report on Tabes.) 

Symptoms. — The symptomatology of tabes is so compre- 
hensive that in order to get a clearer idea of it we shall in our 
description separate the cerebral from the spinal symptoms. 

The cerebral symptoms which appear in the course of the 

629 



630 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

disease are referable either to the cranial nerves or to the brain 
substance. When the latter is affected, it is sometimes the 
cortex, at other times the white substance, or again the basal 
ganglia, which are most deeply implicated. 

In considering the cranial nerves, we shall find that there is 
hardly a single pair which can not be affected and in which 
lesions have not been repeatedly described in cases of tabes. 
However, as we shall see later, not all of them are implicated 
with the same frequency. Among them the nerves supplying 
the muscles of the eye are most commonly, the facial most 
rarely, attacked. Between these extremes we may put in de- 
scending order the vagus, the optic, the fifth, the olfactory, the 
glossopharyngeal, the accessorius, the hypoglossal, and lastly 
the auditory. I have observed three cases in which several 
pairs of nerves were involved at the same time and in which 
the onset of the disorder was somewhat acute. 

The lesions of the olfactory nerve possess no great prac- 
tical significance, and it is not quite certain that they are not 
more frequent than is generally supposed. They consist of a 
weakening or even total loss of the sense of smell, or in the 
perception of peculiar, often disgusting odors, as we have 
shown on page 26. We do not know whether these changes 
are due to anatomical lesions or only to some functional dis- 
turbances, and but little is known about the course of such dis- 
orders of the sense of smell. Occasionally, when examining 
into the condition of the sense of taste, one may accidentally 
discover an affection of the sense of smell without being able 
to ascertain how long it has already existed, as it can easily 
have escaped the notice of the patient. Only those who use 
tobacco or snuff perceive the defect very early and appeal to 
a physician for advice and help. Unfortunately, we can do 
but little. The treatment of these affections has been dealt 
with on page 27. 

The most frequent lesion of the optic nerve in tabes is 
atrophy or gray degeneration. Usually both eyes become af- 
fected, if not simultaneously, at least within a short time of 
each other, and it is quite rare for one eye to be diseased while 
the other remains healthy for any great length of time. The 
patients complain that everything seems as if covered by a 
gray veil. The loss of vision is particularly rapid at first ; it 
then becomes much slower, and the complete amaurosis occurs 
much later than one would have expected from the brusque 



TABES DORSALIS. 63 1 

onset of the trouble. Along- with this, a narrowing (not al- 
ways concentric) of the visual field appears, as the peripheral 
portions of the retina are the first to become impaired in their 
functions. The perception for color may also be affected, as 
we pointed out on page 34. The order in which these changes 
occur is not always the same. As a rule, however, the loss of 
color perception and the narrowing of the visual field precede 
the lessening in acuteness of the central vision, and it is excep- 
tional to find diminished acuteness of vision and marked dis- 
turbance of color sense combined with a normal visual field. 

With regard to the frequency of the affection of the optic 
nerve in tabes, the usual statements of authors hardly give a 
correct idea ; the more careful our examinations are the more 
often do we find them. According to my experience, it may 
certainly be said that they occur in sixty per cent of all cases 
(cf. the excellent piece of work of Martin, De I'atrophie du nerf 
optique et sa valeur prognostique dans la sclerose des cordons 
posterieurs de la moelle, Paris, Asselin et Houzeau, 1890). 

The ophthalmoscopic examination shows a pale grayish 
white or bluish white, but not pure white, discoloration of the 
disk, which is thought to be produced by the obliteration of 
numerous fine vessels in the optic nerve. When the ambly- 
opia is marked, but no perceptible changes in the disk are 
found, we must think of a retrobulbar degeneration of the optic 
nerve. From a pathological standpoint we are dealing with a 
degenerative atrophy, first of the medullary sheaths, and then 
of the axis cylinders. The theory that these changes are due 
to an action of the sympathetic nerves or to changes in the 
vaso-motor nerves brought about by the spinal disease is quite 
untenable, for the process is a neuritis in which we have a 
wasting of the nerve fibres and changes in the interstitial tis- 
sue, such as have been described on page 331. 

For the optic atrophy the outlook is altogether unfavor- 
able ; although a slight improvement or a temporary arrest of 
its progress may give the patient a delusive hope of recovery, 
the termination is always in total blindness. It is true that 
the process may take several years, during which the patient 
is still able to find his way about by himself with the aid of a 
stick. 

With such a prognosis we shall not be surprised if the 
treatment is without avail. The subcutaneous injections of 
strychnine, one milligramme (grs. J / 6o ) twice daily in the neigh- 



632 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

borhood of the eye, as proposed by some, are of value only 
because they give the patient the comforting satisfaction that 
something is being done for him, but they really have no cura- 
tive properties, and it is improbable that they even postpone 
the unfavorable issue. 

In a few isolated cases transient lachrymation has been ob- 
served (Patrolacci, These de Montpellier, 1886; Fere, L'Ence- 
phale, 1887, y ii> 4)- 

The nerves which supply the eye muscles — the third, the 
patheticus, and the abducens — the affections of which have al- 
ready been considered in Part II, Chapter III, frequently be- 
come attacked in the course of tabes. Besides the insuffi- 
ciency of convergence, the central form of which may be 
termed motor asthenopia (Hiibscher, Deutsche med. Wo- 
chenschr., 1892, 17), one often encounters a diplopia resulting 
from a paralysis of the ocular muscles. This may appear sud- 
denly, and after a longer or shorter duration disappear as 
quickly ; or, again, it may recur repeatedly and be a source of 
great annoyance to the patient in his daily occupations. An 
abducens paralysis may also occur by itself, and, finding this, 
one should always look for a commencing tabes, for it is fre- 
quently the first sign of this disease in an apparently quite 
healthy person. If the affection remains stationary, it is to be 
regarded as being due to a nuclear lesion ; the same remarks 
apply to a ptosis which, occurring by itself, is also a suspicious 
sism, and should lead us to look for tabes. In cases of oculo- 
motor paralysis the lesion is also relatively frequently nuclear 
(page 46). Watteville (Neurol. Centralbl., 1887, 10) has called 
attention to a paralysis of the movements of convergence, espe- 
cially in the initial stages of tabes. Borel, in a paper published 
under the direction of Landolt in Paris (Arch. f. Ophthalm., 
Novembre, 1887), has dealt with the same symptoms. Several 
of the extrinsic eye muscles may be affected at the same time, 
and an ophthalmoplegia externa is not infrequently observed 
in the course of tabes. 

The behavior of the intrinsic eye muscles is not less inter- 
esting, and the condition of the pupils deserves the most thor- 
ough examination ; they are rarely normal and of the same size 
in both eyes. Frequently some abnormity of reaction is de- 
monstrable ; the marked contraction (myosis), the difference in 
the size of the two pupils (anisocoria), and the loss of the light 
reflex have already been mentioned. These changes force 



TABES DORSALIS. 633 

us to assume a lesion in the floor of the fourth ventricle 
(Guillery). 

The ophthalmoplegia interna of Hutchinson, in which be- 
sides the loss of the light reflex there is also paralysis of the 
muscles of accommodation, is much rarer. The pupils of those 
afflicted with tabes may frequently be found to dilate promptly 
and normally under strong and painful irritation of the skin, as, 
for example, that produced with the faradic brush. 

The role which the affections of the fifth nerve play in this 
disease is quite subordinate ; paralytic conditions of either its 
motor or sensory branches as the result of tabes have, it seems, 
never been observed except in Westphal's case, in which there 
was degeneration of the ascending root of the fifth, and among 
the signs of irritation only the headache, traceable to the nerve 
endings in the dura, is occasionally met with. A certain rela- 
tionship is said to exist between tabes and genuine migraine, 
but in considering these cases one must make sure that the mi- 
graine has not been inherited, and furthermore note whether 
the attacks become more or less severe after the development 
of the tabetic symptoms. According to some observations, in 
such cases the headache of the migraine becomes less and less 
severe, and eventually disappears, while the nausea and vomit- 
ing still persist, so that it is then impossible to say whether we 
are dealing with a gastric crisis of tabes or with an abortive 
attack of migraine. In certain cases of hemicrania, if there has 
been, for instance, a syphilitic infection at some previous time, 
it is always well to examine carefully for any traces of tabes, 
more especially for the absence of the patellar reflex. Occa- 
sionally one meets with parassthesias in the face, the patient 
complaining of a sensation as if one half of the face and the lips 
were swollen ; this is probably also due to an affection of the 
ascending root of the fifth nerve. 

Lesions of the facial nerve are so rare in tabes that, when 
they occur, one can not help raising the question whether they 
are not to be regarded merely as accidental complications. 
Among three hundred and forty-five cases of locomotor ataxia, 
I have observed only two in which any of the muscles supplied 
by the facial were affected. 

About the same may be said of the auditory nerve. There 
is no question but that lesions of this nerve may be caused by 
tabes or develop in the course of the disease, but they are very 
rare indeed ; they manifest themselves by a diminution or a 



634 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

total loss of the power of hearing-. The patients complain of 
deafness, which may have developed gradually or have come 
on acutely. In both cases the symptoms are due to organic 
disease of the nerve ; in the former we have to deal with a 
gradual gray degeneration of the nerve trunk, in the latter 
with a nuclear affection. Too few cases, however, of involve- 
ment of the auditory nerves in tabes have as yet been ob- 
served to enable us to speak with much certainty of their pa- 
thology (Hermet, L'Union med., 1884, 86; Morpurgo, Arch. f. 
Obrenhlk., 1891, xxx, 26). Under what conditions the so-called 
Meniere's symptoms appear in the course of tabes requires to 
be studied more closely. I have seen them in two of my cases, 
but they disappeared again in a few weeks, and in these cases, 
unfortunately, no anatomical examination of the internal ear 
could be made. 

Functional disturbances of the nerve of taste have now and 
then been described in the course of tabes. In a few instances 
as in the case of Erben, which we considered on page 108, the 
nucleus of the glosso-pharyngeal nerve was degenerated, and 
during life such derangements of the sense of taste existed that 
the patient was at times unable to distinguish sweet things 
from those which were acid or salty. To these lesions no great 
practical significance can be attached. 

On the other hand, there is a great variety of manifesta- 
tions associated with tabes which are due to lesions of the 
vagus. In this connection we have disturbances more gener- 
ally of the digestive, but also of the respiratory and circulatory 
organs. They occur with irregularity, and may disappear 
again quite suddenly. Following the suggestion of Charcot, 
we designate them " crises." Of those affecting the digestive 
system the so-called " pharyngeal crises " are relatively the 
rarest. These consist of paroxysmal movements of degluti- 
tion, which occur from twenty to twenty-four times a minute, 
and succeed one another in this way for ten or twenty minutes ; 
the attacks may be associated with a noisy inspiration, and 
may suggest hysterical singultus ; in some cases they can be 
produced at will by pressure on the side of the larynx (Oppen- 
heim). 

The gastric crises (Charcot) are far more frequent ; they 
consist of paroxysmal attacks of retching and vomiting, during 
which the patient, without any particular exertion, may vomit 
large quantities of strongly acid, slimy, or watery material, some- 



TABES DOR SA LIS. 



635 



times of a blackish appearance, after which he feels greatly re- 
lieved. These attacks are repeated for several days, sometimes 
for a week or two, once, twice, or even oftener, every day, and 
then disappear entirely for a longer or shorter period. In 
some cases the vomiting is associated with cardialgia, but usu- 
ally it is uncomplicated. It is not at all connected with the 
taking of food ; indeed, it not infrequently occurs early in the 
morning when the stomach is empty, and if the patient be a 
drinking man it may arouse a suspicion of the morning vomit- 
ing of drunkards. The differential diagnosis is, however, not 
at all difficult ; if the vomiting be associated with vertigo, a 
sensation of anxiety, and a quickening of the pulse, it can not 
be considered simply as a "gastric crisis." This paroxysmal 
vomiting is of the greatest importance for the diagnosis of 
tabes. It is frequently regarded as dependent upon some 
stomach trouble and treated as such for a long period without 
any sign of improvement, until finally, perhaps by accident, 
our attention is drawn to some other symptom which places 
the diagnosis beyond doubt. If a person have paroxysmal 
vomiting and complain occasionally of violent rheumatoid pains 
in the legs, we should examine most carefully for tabes, and we 
shall frequently be surprised at the ease with which we can 
make a diagnosis, and wonder that we had ever been under the 
impression that the patient had simply " chronic gastritis " and 
" rheumatism." The statement of Eckert (Die intestinalen 
Erscheinungen der Tabes, Inaug.-Diss., Berlin, 1887) that gas- 
tric crises must be divided into those of central and those of 
reflex origin deserves to be investigated more closely. In the 
central form he assumes, besides a general condition of irrita- 
tion in the brain, some affection of the nucleus of the vagus, in 
the reflex form a peripheral irritation of the vagus which, 
under certain circumstances, may be produced by the ingesta. 
He holds that in the latter cases the vomiting is not associated 
with any distressing nausea, so that the patient suffers rela- 
tively little. 

Sometimes intestinal disturbances manifest themselves by 
intense " lightning " pains about the rectum and anus, the " anal 
crises " ; in other cases by tenesmus, which forces the patient 
to go frequently to stool, though he is able to pass little or 
nothing ; and lastly by the so-called tabetic diarrhoea, about the 
causation of which we are absolutely ignorant. This diarrhoea 
may be more or less persistent, and be followed by an equally 



636 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

protracted and obstinate constipation. Incontinence of fasces 
is rarely present, though on rectal examination we shall occa- 
sionally discover sensory disturbances, particularly anaesthesia 
of the mucous membrane. Paresthesias may also occur, and 
the patient may experience a sensation as if he had a foreign 
body in the rectum. 

By " laryngeal crises " we mean those paroxysms of dysp- 
noea which may occur when the patient is lying down, or, in 
other cases, only when he attempts to move or walk about. 
Sometimes they appear in the form of peculiar suffocative at- 
tacks, accompanied by violent coughing, and are often pre- 
ceded by a sighing or whistling inspiration. These attacks 
may last several minutes, during which the suffering may be 
so intense that the patient gives up ail hopes of recovery. 
Attacks of even moderate intensity, in which a long, sonorous 
inspiration follows several short expirations, are most disagree- 
able for the patient, and appear very serious ; under some cir- 
cumstances they may be mistaken for whooping-cough. These 
crises are caused by changes of temperature, speaking for a 
long time, or by strong odors, smoking, etc. The result of the 
laryngoscopic examination is frequently negative ; in other 
cases one finds paralysis of some of the laryngeal muscles ; 
here also, in all probability, we should distinguish a central 
and a reflex form. 

Abductor paralysis — i. e., paralysis of the muscles that open 
the glottis — sometimes occurs among the early signs of tabes, 
and may lead to serious danger of suffocation ; but we are 
unable to say whether this should be attributed exclusively to 
paralysis of the abductors, or to spasm of the adductors alone, 
or to both conditions. We may consider the condition de- 
scribed by Gray (Brain, January, 1888), in which the voice 
often breaks and takes on a high falsetto, as a kind of " laryngeal 
ataxia." 

Attacks of angina pectoris, with all its characteristic symp- 
toms, are rarely met with in tabes, though Vulpian, among 
others, has seen them (Revue de med., 1885, v, 1). 

Lesions of the accessorius are considered as rarities in the 
course of tabes. They are occasionally found associated with 
a posticus paralysis when the outer branch of the spinal acces- 
sory is also affected ; the sterno-cleido-mastoid and trapezius 
muscles then show atrophic changes. In a case observed by 
Martius there was an atrophic paralysis of the upper portions 



TABES DORSALIS. 



637 



of both trapezii, while the sterno-cleido-mastoids, which also 
receive fibres from the cervical plexus, were not affected. 
Whether, and if so under what circumstances, one or both of 
the two nuclei of the accessory nerve are affected (the nucleus 
accessorius vagi and the nucleus spinalis) we are absolutely 
ignorant. It is also uncertain whether symptoms of irritation 
in the domain of the accessorius — e. g., torticollis — ever occur 
in the course of tabes. 

Among the lesions of the hypoglossus there is one which 
deserves a special mention in this place — that is, the hemi- 
atrophy of the tongue described above, which Ballet (lit. page 
144) stated was relatively often observed. He even went so 
far as to say that, when one found this hemiatrophy, tabes 




Fig. 170.— Hemiatrophy of the Tongue in an otherwise perfectly Healthy 
Child (personal observation). 

should always be suspected. We can only agree with him to 
a limited extent. We have certainly found hemiatrophy in 
cases of tabes, but one should remember that it is in itself a rare 
affection, and that it exists more often independently than asso- 
ciated with tabes. In addition to the two cases mentioned on 



638 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

pages 143 and 144 I have recorded another (Fig-. 170) in which 
there was likewise no trace of locomotor ataxia. It seems that 
the hypoglossal nucleus is not very liable to the degenerative 
processes of this disease. 

Cerebral disturbances of the most manifold variety appear 
in the course of tabes, and in the first place attention must be 
called to the paroxysms of vertigo which come over the pa- 
tient when he looks up or makes quick movements of the head, 
and which impel him to seize the nearest object to prevent 
himself from falling. There may also be found psychical de- 
pression and a feeling of dread and anxiety, which in some 
cases may be followed by well-marked psychoses. Among the 
not very rare forms of psychoses in this disease we may men- 
tion paranoia, melancholia, and simple dementia ; but far more 
frequent and important than all these taken together is gen- 
eral paralysis, which very frequently accompanies tabes. But 
here we must try to make out which of the two affections was 
the first to develop, for in some instances the tabes precedes the 
paralysis, while in others the reverse is the case. The process 
can extend from the brain to the cord or from the cord to the 
brain, as the case may be, and Westphal was certainly justified 
in making the statement that " in certain persons there is a 
peculiar disposition of the nervous system, and that this, under 
the influence of different exciting causes, the action of which 
we do not understand, expresses itself in the form of affections 
either of the spinal or cerebral portion of the nervous system or 
of the peripheral cranial nerves, the different affections coming 
on in some cases nearly at the same time, in other cases at 
varying intervals." 

Epilepsy occurring in connection with tabes has already 
been considered in the chapter on the former disease. On this 
subject Schlieper, working under my direction, has published 
an article (Inaug.-Diss., Breslau, 1884). 

The cases of hemiplegia which occur in the course of tabes 
are mostly of the indirect variety — that is, they disappear in a 
shorter or longer time — and do not owe their origin to the 
rupture of vessels or to lesions of the internal capsule. The 
face is usually only slightly affected, and that only for a short 
time, and the extremities are not wholly paralyzed, but are 
only in a paretic condition, which usually disappears without 
any sort of treatment. I have repeatedly seen such cases of 
hemiparesis come on without any warning and with only a 



TABES DORSALIS. 



639 



slight disturbance of consciousness and entirely disappear in 
a relatively short time. A. Bernhardt (Archiv f. Psych. 11. 
Nervenkrankheiten, 1883, xiv, 1) has recorded instances in 
which they were accompanied by aphasic conditions. 

LITERATURE. 
2. The Brain and the Cranial A r erz'es. 

Berger. Des troubles oculaires dans le tabes, etc. Revue de med., 1890, 3. 

Schultze. Arch. f. Psych., 1889, xxi, 2. (Paralysis of the Muscles of Mastica- 
tion.) 

Chataigner. Des troubles auditifs dans le tabes. These de Paris, 1889. 

Marina. Arch. f. Psych., 1891, xxi, 1. (Symptoms referable to the Ear and 
Larynx.) 

Minor. Zeitschr. f. klin. Med., 1891, 5, 6. (Hemiplegia, etc.) 

Charbert. Cas de tabes a debut cephalique caracterise par la lesion des 2 mc9 , 
3-, 4-, 5- et 6 mes paires craniennes. Progres med., 1892, 20. 

Guillery. Ueber die topische Diagnostik der Pupillarerscheinungen bei der 
Tabes. Deutsche med. Wochenschr., 1892, 52. 

Chvostek. Tabes mit Bulbarsymptomen. Neurol. Centralbl., 1893,22. 

Eulenburg. Ueber einige Falle von Tabes mit Betheiligung des Vagus und 
Accessorius. Inaug.-Dissert., Berlin, 1893. 

Ilberg. Charite-Annalen, 1893, p. 303. (Accessorius Paralysis.) 

Laffitte. Des Crises gastriques. Gaz. des hop., 1894, 3. 

So great importance has been attached to the spinal symp- 
toms that they usually occupy the greater part of all descrip- 
tions of the clinical history of the affection, and have been 
allowed to predominate so far that all other symptoms have 
been treated of as being of little moment, and as if the only 
lesion was that in the spinal cord. And still, it is not rare to 
meet with cases in which the spinal manifestations have been 
for a long period of very little importance, and with a few in 
which they have never attained to any prominence, while the 
majority of the troublesome symptoms were due to affections 
of the brain and its nerves, and the lesions of the peripheral 
nerves gave rise to more marked symptoms than those of the 
spinal cord. Observations of this kind, the number of which 
will be rapidly increased by conscientious examinations, go to 
show that the entire nervous system participates in the morbid 
process, and to consider this participation to be the rule is 
absolutely necessary for a correct comprehension of the pathol- 
ogy of this disease. 

The symptoms produced by the spinal lesions concern mo- 
tility, sensibility, and the reflexes. 



640 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

The disturbances of motility are manifold ; they depend 
partly on a decrease in the strength of the muscles, partly on 
disturbances of co-ordination. The first is not very common ; 
on the contrary, one can frequently observe that the mere 
strength in the extremities has not been at all affected, and 
yet the motility has suffered. This condition depends, then, 
upon a faulty co-ordination, and is broadly designated as 
" ataxia." Movements, such as walking, writing, taking hold 
of an object, etc., for the proper execution of which the simul- 
taneous working together of several muscles is necessary, are 
designated as " co-ordinated." For such movements more than 
a simple innervation of the muscles is requisite ; it is necessary 
that each concerned should receive, so to speak, the proper 
amount of innervation and at the proper time, so that the 
contraction of the various muscles may take place at the right 
moment. It is only when all these various factors are prop- 
erly combined that the movement is correctly executed, and if 
one of them be disturbed the entire movement becomes ataxic. 
Even if not pathognomonic, it is certainly very characteristic 
of tabes that in the later (rarely in the earliest) stages, certain 
movements become ataxic, particularly those of the lower ex- 
tremities, and, above all, the gait. Such abnormities are met 
with much less frequently in the upper extremities, and the 
movements necessary for writing, handling a spoon in eating, 
and the like, usually remain normal. 

The gait of a tabetic is readily recognized even by one who 
has had little experience in that direction ; one notices particu- 
larly that the patient exerts his eyes almost as much as his feet, 
that he watches every step, and in passing over small obstacles, 
as for example a curbstone, determines exactly where he must 
place his foot. If he ceases to use his eyes in this fashion for 
any reason, even for a short time, the movements of the legs 
become uncertain, and he is in danger of falling. But not even 
with the help of the eyes can he walk without difficulty. He 
does not step out in the usual way ; the legs are thrown out 
loosely, and in putting the feet to the ground the heels come 
down first (" strutting gait "). The manner in which the feet 
are raised, the legs thrown out, the stamp with which the feet 
touch the ground, readily enable one to diagnosticate the ta- 
betic gait at a distance, and we shall seldom make a mistake if 
we consider a person who walks in this manner, supported on 
a stick or by an attendant, as affected with locomotor ataxia. 



TABES DORS A LIS, 



641 



Acts of politeness, such as greetings and stopping to talk on 
the street, do not afford these persons much pleasure, for they 
distract their attention, which has to be kept undivided if they 
would walk in safety. 

The uncertainty and insufficiency of the innervation of the 
different groups of muscles is apparent not only in the walk, 
but even while the patient is standing still. He is not able to 
stand up straight without tottering, particularly when he closes 
his eyes, and he sways to and fro and falls unless some one is 
at hand to support him (" Romberg's sign "). The smaller the 
supporting basis — that is, the nearer together the feet— the 
more pronounced does the phenomenon become. In some 
cases it may be accompanied by irregular contractions of the 
calf muscles. 

The much rarer ataxia of the upper extremities produces 
inability to write, to play the piano, to sew, etc. With closed 
eyes the patient is unable to describe circles in the air with his 
arms, to bring the tips of the index fingers together from a dis- 
tance, or to touch the end of the nose quickly with his finger. 
All such movements are carried out with more or less irregu- 
larity. It is exceptional for the upper extremities to become 
affected at an early period or severely ; as a rule, we can not 
detect ataxic movements in them in the earlier stages, and when 
they do occur they can, at least in some instances, be traced to 
some special cause. In the case of Bernhardt (Zeitschr. f. klin. 
Med., 1888, xiv, 3, p. 289) they were due to the occupation of 
the patient. Remak (Berlin, klin. Wochenschr., 1880, 22) has 
also published a similar case of ataxia affecting only the upper 
extremities. It was associated with ephidrosis unilateralis. The 
helplessness of the patient reaches the most extreme degree 
when the ataxia affects all four extremities, as in the case of 
Fort (Dublin Journal of Medical Science, 3d s., 1886, clxxiii). 

But we must also distinguish between spinal and cerebral 
or the so-called cortical ataxia (page 186). A conclusion im- 
portant for the differential diagnosis may be drawn from 
observing the influence which the eyes exert over the co-ordi- 
nated movements. In spinal ataxia these become better regu- 
lated and more certain when they are under the control of the 
eyes, while in cortical ataxia this factor has no influence. 

The physiological cause of ataxia is not as yet positively 
known, but even to-day is a source of contention and still the 
object of continued investigations. While some, as Benedikt, 
41 



642 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Cyon, and Jaccoud, consider that we have to do with a disturb- 
ance of the reflex activity in the cord, others, with Friedreich, 
and after him Erb, are of the opinion that there is a disturbance 
in co-ordinating fibres, the course of which they confess can 
not as yet be made out. Thirdly, others, with Leyden at 
their head, consider disturbances of sensibility to be respon- 
sible for the ataxia. According to these, interruption of con- 
duction in the sensory tracts of the gray matter causes a break 
of the reflex arc between the sensory nerves of the muscles 
and the motor nerves. " Owing to this interruption, the un- 
conscious regulation of the movements, which adapts them 
to the state of contraction or relaxation of the musculature, 
disappears" (Wernicke), and ataxia is the result. This " sen- 
sory ataxia " has always had many opponents, for one was 
obliged to confess that ataxia often occurs when no sen- 
sory changes are found ; but in spite of this fact some one is 
constantly returning to this theory, which has found a strong 
advocate in Goldscheider. In a comprehensive article (Zeit- 
schr. f. klin. Med., 1888, xv, 1, 2) he subjects the meaning of the 
term " muscular sense " to a fresh examination, and comes to 
the conclusion that four factors are combined in the formation 
of the muscular sense, viz.: (1) the sensibility to active, (2) to 
passive movements, (3) the perception of position, and (4) the 
perception of weight and resistance. He then states that in 
all cases of ataxia in which the sensibility had been tested the 
examination had been imperfect in some detail ; he points out 
that, for example, in the otherwise admirably conducted 
observations of Friedreich, the examination of the sensibility 
to movement was omitted. According to his view, therefore, 
it is only necessary to perfect the examination of the sensibility 
in order to come to the conclusion that sensory disturbances 
are responsible for the ataxia. 

When one considers that we are ignorant of the origin of 
the normal co-ordination, and remembers that it is not congeni- 
tal but must be learned by practice, in which controlling and 
correcting influences, which arise from the periphery, come 
into play, it is not difficult to agree with Striimpell, who con- 
siders that the ataxia takes its origin from the disappearance 
or insufficiency of those regulating influences, because " the 
possibility of successfully transferring them to the motor ap- 
paratus is removed." We should then have to regard the gray 
substance and the ganglionic cells as the place where this 



TABES DORS A LIS. 



643 



transfer probably occurs. Which of the theories above men- 
tioned will at last be recognized as the correct one, and whether 
or not other factors, which have not yet been considered, play 
a part in the production of the ataxia, it is at present impossible 
to state (cf. Rumpf, Sensibilitatsstorungen und Ataxie, Leipzig, 
Hirschwald, 1889). 

Later on in the course of tabes there is a diminution in the 
actual strength of the voluntary muscles, particularly in those 
of the lower extremities. This first manifests itself by wea- 
riness on walking, which gradually increases, and finally ends 
in total paralysis (paraplegia). The patient first notices that 
he has to rest in the course of walks which he previously 
was in the habit of taking without any feeling of fatigue, that 








Fig. 171. — Specimen of Handwriting in a Case of Tremor in Tabes 
(personal observation). 

it takes him much longer than formerly to cover a particular 
distance, and that he is in general unable to take the exercise 
to which he was formerly accustomed. As the disease advances, 
the power of locomotion becomes more and more diminished, 
and the patient is only just able to drag his legs along, and 
at last, becoming unable to move at all, or even stand without 
help, is obliged to spend the rest of his life in the invalid's 
chair. 

Signs of motor irritation are rare and are limited to parox- 
ysmal twitchings in the fingers and toes ; sometimes, however, 
involuntary movements occur in the limbs which the patient 



644 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

has absolutely no intention of moving. Stintzing (Centralb. f. 
Nervenheilk., 1886,9, 3), for example, observed an involuntary 
flexion of the hip joint when the patient coughed. Similar 
associated movements in the fingers or toes have been de- 
scribed by Strumpell (Neurol. Centralblatt, 1887, vi, 1) and 
Oppenheim (Sitzung der Charite-Gesellschalt, 20 Marz, 1884). 

The athetoid and choreiform movements described by 
Andry (Revue de med., 1887, 1), sometimes found in tabetics, 
are to be regarded as due to simultaneous disease of the lateral 
columns, and accordingly rather as complications. We must 
regard the tremor as one of the signs of motor irritation, al- 
though we are at present unable to localize its anatomical seat. 
This symptom is sometimes observed either in the initial stage 
or in the further course of the disease. If the upper extremities 
become affected by it, the handwriting is altered, in the man- 
ner represented in Fig. 171. 

The disturbances of sensibility in tabes are either experi- 
enced subjectively by the patient, or can only be discovered 
by an objective examination. Their number is exceedingly 
large, and it is safe to say that in almost every case some inter- 
esting observation of this character may be made. Symptoms 
of irritation alternate with those of paralysis, and one also 
meets with other different disturbances of sensation which be- 
long to neither of these groups, and which are more variable 
in tabes than in any other affection. 

Among the subjective symptoms we shall consider first the 
symptoms of irritation, more particularly the pains, which in 
the life of tabetics play such an important part. They, too, 
are of a changeable nature, and vary considerably in their 
situation and intensity. In the first place we desire to direct 
attention to the muscular pains, which, if they occur at all, 
come on very early in the course of the disease, and affect 
sometimes the shoulders, sometimes the legs, and recall the 
well-known muscular pains which follow severe exertion in 
the gymnasium, mountain climbing, rowing, etc. As a rule, 
it is true, they are not very intense, but when they come 
on suddenly, without any appreciable cause, the patient is 
obliged to remain perfectly quiet for several hours, for every 
motion is difficult to him, and if he persists in his attempts, 
movement becomes impossible on account of the feeling of 
weakness and fatigue which at last overcomes him. Pitres 
calls these pains " crises de courbature musculaire " (Progr. 



TABES DOR SA LIS. 645 

med., 1884, xii, 28), and considers that they are precursors of 
tabes. 

We must separate from these the nervous pains of tabetics 
which are dependent upon irritation of the posterior roots. 
They are usually situated in the lower extremities, and mani- 
fest themselves either as dull, boring sensations, or as sharp 
pains which last for hours and then disappear for a time ; they 
may also be felt in the back and sacral region, and for years be 
attributed to rheumatism, lumbago, etc. As long as only these 
pains exist, the life of the patient is bearable, although it may 
be marred and his occupation interfered with, but there is a 
second class of nervous pains which, appearing and disappear- 
ing like lightning, are known as shooting or lancinating pains, 
" douleurs fulgur antes." It is these that make the existence of 
the tabetic most miserable, and make him wish that he were 
dead ; it is these, again, that can reach an intensity which 
causes the most resolute sufferer to lose his energy, and con- 
verts him into a complaining and whining weakling. They 
also occur paroxysmally, and may continue for minutes, hours, 
or even days, and then disappear for variable periods, some- 
times for months. In many cases they recur often, some- 
times every week, but they then usually only last for a few 
moments. 

In some cases, in connection with these attacks, cutaneous 
ecchymoses may develop, which are to be noted in the por- 
tions of the body subjected to the pain, and may attain a 
considerable size, so that one who does not know their sig- 
nificance, on examining the patient, may come to the conclu- 
sion that he has been injured by a blow or a fall. In still 
rarer instances swellings have been observed instead of the 
ecchymoses, which in the same manner as the latter disappear 
in a few days. 

Along with these pains the patient may suffer with hyper- 
assthesias of the skin to such an extent that in certain parts of 
the body — very frequently, for instance, on the back — he can 
not bear the slightest pressure, and even his clothes will be a 
source of annoyance to him. These cutaneous hyperesthesias 
may persist for months unchanged without being affected in 
the least by the paroxysmal pains. 

Among the symptoms of sensory irritation the so-called 
girdle sensation may also be reckoned. This likewise occurs 
paroxysmally, at which times the patient experiences a feeling 



646 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

as if a belt were being drawn around his chest and abdomen, 
which interferes with his breathing. 

Manifestations of sensory paralysis may also be subjectively 
perceived by the patient. Not infrequently he will say that 
he does not feel the contact of the clothing on certain portions 
of the body, or that the soles of the feet are without sensation. 
In a case under my observation the patient complained of a 
widespread loss of sensation in the perineal region, which on 
objective examination proved to be anaesthetic as well as the 
inner surfaces of both thighs. To the anaesthesias, which are 
particularly unpleasant to the patient, belong those affecting 
the mucous membranes — as, for example, that of the rectum — 
owing to which the bowel may empty itself without the patient 
being conscious of it. Again, there may be anaesthesia of the 
testicle, often associated with atrophy (Pitres), and loss of sen- 
sation in the mucous membrane of the sexual organs — as of the 
vagina, for example — owing to which the pleasurable sensa- 
tions attending coitus are either absent or greatly diminished. 

Among the perverted sensations which are experienced 
subjectively may be mentioned the alterations of feeling in the 
soles of the feet, owing to which it appears to the patient that 
he is not walking upon solid ground, but rather upon a soft 
yielding surface, such as moss, cotton, etc. To these may be 
added the sensation as of ants crawling over the skin, a feeling 
of numbness, which usually appears in the lower extremities, 
but sometimes also in the hands. In the latter case it may 
become impossible for the person to write, sew, etc., in spite 
of the fact that he may be suffering from no disturbance of 
motility whatsoever. 

Many anomalies of sensation in tabes can only be discov- 
ered by means of objective examination. They constitute the 
second group of sensory disturbances to which we referred 
above. 

We would here insist upon the necessity of making the 
examination as carefully as possible, and of remembering in 
the first place that when the patient is repeatedly examined he 
leases to give us his attention and makes careless answers to 
the inquiries made of him ; and in the second place that there 
are certain sensations, the so-called spontaneous sensations, 
which the patient experiences without any external irritation 
whatever. Rosenbach (Deutsche med. Wochenschr., 1889, 13) 
holds that accumulations of weak sensory stimuli occur, the 



TABES DORSALIS. 



647 



intervals between which vary according to the strength of the 
stimuli and the better or worse condition of the patient. If 
one remembers this and the fact that the so called after-sensa- 
tions must also be taken into account when making the test, 
one will be able to avoid gross errors. B. Stern (Arch. f. 
Psych, und Nervenkrankheiten, 1886, xvii, 2) has not been able 
to confirm the statement of Belmont (Gaz. med., 1877, 19) that 
points of predilection exist for the disturbances of sensation in 
tabetics, as, for example, in the soles of the feet, the areas about 
the malleoli, and the lower extremities in general. Were it 
true, it might constitute a new source of error in the examina- 
tion of the anomalies of sensation. The methods of examina- 
tion are as simple as possible, and the necessary instruments 
are an induction apparatus, Weber's aesthesiometer, needles, 
mounted brushes, and test tubes filled with hot and cold water. 
With these one is able in most cases to obtain all the necessary 
information. 

Among the symptoms of irritation, hyperesthesias, as we 
stated above, are not of very frequent occurrence, but when 
they do occur they can very easily be recognized. They are 
frequently quite transient, so that a point, which yesterday was 
sensitive to the slightest touch, presents to-day a perfectly 
normal condition. The exaggerated sensitiveness is probably 
always confined to the perception of pain, but is not found 
associated with the other qualities of sensation. We recognize 
another symptom of irritation in the so-called double percep 
tion of painful impressions, polyassthesia (Fischer), by which 
is meant that from one external irritation, as the prick of a 
needle, the patient experiences two painful sensations in suc- 
cession. 

In the objective examination of the sensibility the symptoms 
of paralysis play, without doubt, the more important role. In 
the first place there are the anaesthesias, which may affect all 
qualities of sensation, the sense of pain, touch, and temperature. 
The most interesting is an analgesia, to which Berger first 
directed attention, who demonstrated that while the patients 
reacted normally to slight stimuli, they scarcely did so at all to 
stronger ones. We must consider it as an anomalous analgesia, 
when a patient experiences only one kind of pain in response to 
the most varied kinds of painful stimuli. It sometimes happens 
that the tabetic can not tell the difference between the action 
of the thermo-cautery, the simple prick of a needle, or a violent 



648 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

pinch, and designates the pain produced by these various 
agents as simply a burning one. The painful sensation on 
electrical stimulation may also become abolished, so that we 
can apply the strongest currents or the faradic brush to the 
most sensitive parts, such as the inner surfaces of the thighs, 
the perinseum, or the scrotum, and the patient not give the 
slightest evidence of pain. 

Lastly, delayed sensation is to be considered as a symptom 
of a paralytic nature. In these cases, when the patient is 
pricked with a needle, he does not experience pain until one, 
two, or three seconds later. Goldscheider has attempted a 
physiological explanation of these phenomena (Deutsche Med.- 
Ztg., 1890, 43, p. 484). The delay of perception may vary for 
the different qualities of sensation — for example, for touch and 
pain — as Osthoff and Remak have pointed out. 

We must attribute to disturbances of the muscular sense, 
which we alluded to in discussing the cause of ataxia, the fact 
that the patient with his eyes closed is unable to state accu- 
rately in what position his extremities are, and if one, for in- 
stance, changes the position of a limb, he is not at all certain 
into what position it has been put. He is unable to estimate 
the weight of an object placed in his hands, and so forth. All 
these conditions are to be remembered when one is testing the 
muscular sense, and at the examination one will have to ascer- 
tain what is the minimum change of position which can still be 
recognized by the patient. 



LITERATURE. 

3. The Spinal Cord. 

a. Motor and Sensory Disorders. 

Rumpf. Sensibilkatsstbrungen und Ataxic Deutsches Arch. f. klin. Med., 

1889, 1. 
Goldscheider. Berliner klin. Wochenschr., 1890,46. 
Binswanger. Ibid., 1890, 21, 22. 

Quincke. Zeitschr. f. klin. Med., 1890, 5. (Associated Sensations, etc.) 
Wagner, K. Ueber die Beziehungen der Bewegungsempfindung zur Ataxie bei 

Tabikern. Inaug.-Dissert., Berlin, 1891. 
Stanowski. Versuch, die Ursache der Ataxie bei Tabes zu erklaren. Internat. 

klin. Rundschau, 1892, 17. (This author explains ataxia by a diminution 

in the excitability and conductivity of the motor nerves.) 
Dubuc. Des douleurs fulgurantes tabetiques. These de Bordeaux, 1892. 
Grasset. Du vertige des ataxiques (signe de Romberg). Arch, de Neurol., 

1893, 73> 74- 



TABES DOR SA LIS. 649 

Of the disturbances of the reflexes, those connected with 
the skin interest us less than those connected with the tendons ; 
of the latter, the patellar reflex is the most important, the ana- 
tomical localization for which is in the so-called root zone 
(Westphal). This zone is situated at the junction of the 
lower dorsal portion of the cord with the lumbar enlargement 
at the level of exit of the second, third, and fourth lumbar 
nerves (cf. page 422), and constitutes the area which the 
roots entering to the median side of the posterior horn must 
traverse in order to reach the substantia gelatinosa of the pos- 
terior horn. If this field is degenerated the patella tendon 

A. B 

i 





Fig. 172.— Two Cases of Tabes. (After Westphal.) 

A. The lines hh show the limits of the so-called " root zone.'' The degeneration is progress- 

ing; from within toward them, but only reaches the border line. The patellar reflexes 
were retained until death. 

B. The degeneration is progressing from within outward, and has extended far into the 

" root zone." The patellar reflexes were lost five years before death. 

reflex disappears, but if it is normal, the reflex is preserved 
(cf. Fig. 172, A and B). The rare cases in which it remains 
preserved on one side also confirm the localization assumed 
by Westphal ; at the autopsy it has been repeatedly noted (cf. 
Berlin, klin. Wochenschr., 1887, 31, p. 586) that there was a 
degeneration of the posterior columns and of the "root zone" 
on the affected side, while this zone on the healthy side was 
intact. 

The disappearance of the patellar reflex, " Westphal's sign," 
was formerly considered as pathognomonic of tabes, and when- 
ever the knee jerk could not be obtained, the diagnosis was 
made without hesitation. This was the standpoint taken in 
the earlier works of Westphal, Erb, and others, and it must be 
confessed that " Westphal's sign " is observed in by far the 
greater number of cases of tabes, and usually early in the 
course. However, it began to be doubted that the rule was 



650 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

without exceptions, and toward the end of the seventies sev- 
eral undoubted cases of tabes were reported (Berger, Fournier) 
in which the patellar reflex was retained to the end of life, and, 
since then, other similar cases have been added. Westphal 
himself pointed out that the knee phenomenon might persist 
with degeneration of the posterior columns (Arch. f. Psych, 
und Nervenkrankh., 1886, 17, 2), and precisely at this time I 
myself reported two such instances (Berlin, klin. Wochenschr., 
1886, 10). Accordingly, it is an undeniable fact, and one 
which, anatomically, can be readily explained, that under cer- 
tain circumstances — that is, whenever the " root zone " remains 
free from degeneration — the patellar reflex may continue to be 
present during the entire course of the disease. By repeated 
and accurate examination, in which Jendrassik's method of 
re-enforcement should not be forgotten, one is sometimes able 
to follow up the gradual disappearance of this reflex, and to 
observe that the time of its diminution and final disappearance 
may differ in the two legs — for example, the reflex may still be 
well marked on one side, after it has completely disappeared 
on the other. Among others, Goldflam has reported observa- 
tions on this point (Neurol. Centralblatt, 1888, 19), and has sup- 
posed that interference with conduction, produced by patho- 
logical changes in the peripheral nerves, may also be the cause. 
Eichhorst has reported a case in which, although the patellar 
reflex had been absent, the autopsy revealed no changes in the 
root zone, but a parenchymatous neuritis of both crural nerves. 
The patellar reflex which has once disappeared in the course of 
tabes can never reappear, since destruction of the correspond- 
ing portions of the cord has taken place, but in traumatic neu- 
roses this may very well happen, and in doubtful instances it 
may become an important point in the differential diagnosis. 
The patellar reflex can only be increased in tabes when there 
is a coincident degeneration of the lateral columns. 

While, then, for the reasons we have given, " Westphal's 
sign " can not be regarded as pathognomonic, there are still 
others which should warn us against laying too much stress on 
the condition of the patellar reflex in the diagnosis of tabes. 
Unquestionably it may also disappear under certain circum- 
stances in the course of other affections — as, for example, in 
certain diseases of the brain — if the muscular tone necessary to 
its production has been lost ; also in neuritis, poliomyelitis, 
diabetes, chronic alcoholism, and in affections of the knee joint 



TABES DORSALIS. 6« r 

when the movements of the tendon are interfered with. When 
we add that it can not be demonstrated in all healthy persons 
— a small number being entirely without it, as Berger and 
others have stated — and moreover consider the fact that in old 
age and in conditions of marked nervous exhaustion it may 
entirely disappear without any apparent reason, perhaps from 
a diminished tone in the muscles, we shall have sufficient 
grounds for not overestimating its significance, important as 
it may still be for the recognition of tabes. The measure- 
ments of its strength, which have lately been made a good 
deal of, may for the present be omitted in practice without 
disadvantage for the diagnosis. 



LITERATURE. 

3. Spinal Cord. 

b. Reflexes. 

Rosenheim. Arch. f. Psych, u. Nervenkh., 1884, xv, 1. (Experimental Studies 

on the " TendoH-phenomena.") 
Zenner. The Knee-phenomenon in Locomotor Ataxia. Journ. of Nerv. and 

Ment Disease, N. S., April, 1884, ix, 2. 
Delprat. Nederld. Tijdschr. voor Geneeskunde, 1886, 51. (Three Cases of 

Tabes in which the Patellar Reflex persisted until within a Few Hours be- 
fore Death.) 
Westphal. Ueber Fortdauer des Kniephanomens bei Degeneration der Hinter- 

strange. Arch. f. Psych, u. Nervenkh., 1886, xvii, 2. 
Mitchell, Weir, and Morris. Tendon-jerk and Muscle-jerk in Diseases, especially 

with Reference to Posterior Sclerosis of the Spinal Cord. New York Med. 

Record, 1886, xxx, 1. 
Krauss, E. Beitrag zur Localisation des Patellarreflexes bei Tabes u. s. w. 

Neurol. Centralbl., 1886, v, 20. 
Hirt. Ueber Tabes mit erhaltenen Patellarreflexen. Berliner klin. Wochenschr., 

1886, xxiii, 10. 
Westphal. Zwei Falle von Tabes mit erhaltenem Kniephanomen. Berliner 

klin. Wochenschr., 1887, xxiv, 5. 
Minor. Zur Frage iiber die Localisation des Patellarreflexes bei Tabes. Cen- 
tralbl. f. Nervenhk., 1887, x, 6. 
Westphal. Anatomischer Befund bei einseitigem Kniephanomen. Arch. f. 

Psych, u. Nervenkh., 1887, xviii, 2. 
Goldflam. U/eber die Ungleichheit des Kniephanomens bei der Tabes. Neurol. 

Centralbl., 1888, vii, 19, 20. 
Warren, Plymton, Lombard. The Variations of the Normal Knee-jerk and 

their Relation to the Activity of the Central Nervous System. Amer. 

Journ. of Psychol., 1887, i, 1. 
Meyer. Berliner klin. Wochenschr., 1888, 2. (Influence of Physostigmine on 

the Tendon Reflexes.) 



652 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Benedikt. Qualitative Veranderungen des Kniephanomens. Neurol. Centralbl., 

1889, 17. 
Eichhorst. Virchow's Arch., 1891, cxxv, 1. (Patellar Reflex in Cervical 

Tabes.) 
Goldflam. Berliner klin. Wochenschr., 1891, 8. (Reappearance of the Patellar 

Reflex.) 
Vucetic, Marie. Inaug.-Dissert., Wien, 1893. 

The reflex centres for the functions ot the bladder, rectum, 
and sexual apparatus, which are situated in the lumbar portion 
of the cord, are naturally also greatly disturbed in the course 
of tabes. The reflex processes, which come into action here, 
are but little understood, but their pathological condition has 
been studied with great care. Much attention has been directed 
toward the bladder troubles of tabetics, and attempts have 
been made to distinguish between the different kinds of affec- 
tions. They are motor or sensory, or both, according as only 
the one or the other or both centres have been destroyed by 
the degenerative process in the cord. 

Among the motor disturbances there are symptoms of irri- 
tation as well as of paralysis, which may affect equally the 
sphincter and the detrusor, so that the will may have but little 
influence over them, or finally none at all. According as one 
or the other condition is the more prominent, the complaints 
ot the patient differ: sometimes he is obliged to strain for a 
long time before the bladder will begin to empty itself, and 
even then the stream is often interrupted ; sometimes he is 
unable to urinate at all in the erect posture, but must squat 
down or sit on the closet to bring the abdominal muscles into 
action in order to expel even a few drops of urine, and the act 
of micturition may take so long that the patient feels ashamed 
to use the public conveniences. In other cases, where there is 
not only paresis of the detrusor, but at the same time a spasm 
of the sphincter, the patient can not urinate at all, and the re- 
tention must be relieved by means of the catheter ; in other 
instances, again, where there is a paresis of the sphincter, he 
has to urinate very frequently. Long before the bladder is 
full — every hour or two — he feels an irresistible desire to empty 
it, which he must satisfy or run the risk of an involuntary pas- 
sage of urine. He is unwilling to undertake railroad journeys, 
to go into society, to lectures, or to the theatre, for fear that 
he will not be able to reach a convenient place in time where 
he can urinate in peace. Paresis of the sphincter is often a 



TABES DORSALIS. 653 

reason why the patient sleeps poorly, because he has to get up 
so often to urinate, and if he sleeps soundly he does not appre- 
ciate the calls of nature, and will pass his urine in bed. When 
he coughs or sneezes the under-garments are moistened with 
urine, and, despite his utmost efforts, he is unable to prevent 
it. In the more marked degrees of weakness of the sphincter 
there is an involuntary trickling or an occasional discharge of 
urine, which the patient is unable to predict ; this necessitates 
the constant wearing of some sort of receptacle ; otherwise 
the patient is surrounded by such an ammoniacal odor that the 
incontinence is recognizable without any examination. If there 
is a combination of retention and incontinence, it manifests it- 
self in the following manner : After long straining the urine is 
passed in a moderately strong stream, but this suddenly ceases, 
and can only be started again after renewed efforts. Some- 
times, after the patient has strained in vain for a long time and 
has given it up in despair, the urine is passed involuntarily. 
These and many other facts of the same description are only 
to be discovered after careful and repeated questionings and 
examinations. 

Sensory disturbances may manifest themselves (1) by more 
or less intense pain before and during the act of micturition, 
which may distress the patient greatly and make him dread to 
relieve his bladder (the " crises ve'sicales '' of Charcot). The 
pain may be situated either in the hypogastric region or ex- 
tend down into the urethra [crises vesico-uretJirales). Painful 
strangury, forcing the patient to urinate every half hour, when 
he only passes a few drops, has also been observed. On the 
other hand, (2) there may be a diminution in sensibility, so 
that, in consequence of the anaesthesia of the mucous mem- 
brane of the bladder and urethra, the flow of urine is not no- 
ticed, and the patients, especially when there is a weakness of 
the sphincter at the same time, do not know whether they are 
urinating or not, and only become aware of the fact when they 
feel the chilly sensation proceeding from the damp clothes. A 
rather rare manifestation, which may be observed after violent 
bladder crises, is the appearance of hasmaturia, which must be 
attributed to capillary haemorrhages into the bladder or ure- 
thra ; the bloody character of the urine may be a source of 
new anxiety and worry to the unfortunate patient, already 
greatly broken down by the agonizing pains. These haemor- 
rhages may be considered as analogous to the ecchymoses in 



654 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

the skin occurring after the intense lancinating pains, which 
we mentioned above on page 645. 

The most troublesome rectal symptom is the very obstinate 
constipation. Incontinentia alvi and anaesthesia of the rectal 




Fig. 173.— A Case of Charcot's Joint in a Tabetic (personal observation). 

mucous membrane, in consequence of which the patient is not 
aware of the act of defecation, and so soils himself uncon- 
sciously, are among the rarer occurrences. 

The centre for the sexual functions, the seat of which is 
also in the lumbar enlargement, is under the control not only 
of reflex but also of cerebral influences. If the path coming 
from the psychical centres is interrupted, the performance of 
the function is faulty ; if the path from the inhibitory centres is 
disturbed, the sexual reflex activity is increased and priapism 
may occur. Pitres (Progr. med., 1884, xii, 37), under the name 
of "crises clitoridiennes" has described in women conditions 



TABES DOKSALIS. 



655 



which consisted of periods of voluptuous excitement accom- 
panied by secretion, analogous to the violent erections and 
spermatorrhoea found in men in the initial stages of tabes. 
Such cases are, however, at least in Germany, exceptional. 
Not infrequently tabetics have been known to preserve their 
virility, and even after the beginning of the disease to beget 
one or even several healthy children. Only later does the 
sexual power, and with it the desire, become diminished, and 
coitus lose its charm, so that it is undertaken more rarely, the 
act being sometimes incomplete. A normal condition of the 
nerves necessary for the erection of the penis, associated with 
a paralysis of those going to the ejaculator seminis, so that 
while coitus and orgasm are normal, the semen is not emitted 
till later, and then very slowly — a condition which Bernhardt 
has observed after injury (Deutsch. Med.-Ztg., 1888, 48) — has 
been known to occur also in the course of tabes. 

The vaso-motor and trophic centres in most cases are not 
affected. In the majority of instances, symptoms of this char- 
acter are entirely absent during the whole course. In some, 
however, peculiar symptoms attract our attention, as, for ex- 
ample, a local hyperidrosis, which Ollivier (Gaz. hebdom., 
Septembre 7, 1883, xxx, 36), Raymond and Arthaud (Revue de 
med., 1884, 4, 5), and others have observed on the hands and 
feet. In a case of tabes we have also seen the sweat secretion 
on the hands so increased that we were able to note the forma- 
tion of small drops and watch them unite to form a steady 
dripping. In another case there was unilateral sweating, the 
hyperidrosis appearing after every meal on the left half of the 
head, face, and neck. I do not care to risk an opinion as to 
how far an assumption of an affection of the sympathetic would 
here be justifiable. 

Greater practical importance must be attributed to the 
changes which are observed in the nails and teeth of those 
affected with tabes. The nails are either deformed, becoming 
twisted or marked by deep furrows, or fall out entirely from 
the fingers as well as from the toes, as Joffroy (L'Union, 1882, 
106), Bonieux (These de Paris, 1883, No. 237), Hay-Margiran- 
diere (These de Paris, 1883, No. 75), and others have observed. 
The loss of the nails (" la chute des ongles ") is not rare in tabes, 
and is in some cases to be attributed to the temporary cessa- 
tion of growth of the nail matrix. In others an ecchymosis 
under the nail may be the exciting cause. Under certain cir- 



656 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

cumstances the nail of the great toe falls off altogether, without 
pain, with only a slight itching sensation, and the newly formed 
nail, which is often rough and irregular, soon shares the fate of 
its predecessor. 

It is occasionally observed that the teeth become loosened 
without any pain and fall out without the appearance of any 
symptoms of inflammation, the tooth itself being intact. This 
arises from some disturbance in the nutrition of the jaw, a rare- 
fying ostitis which is connected with a lesion of the nucleus of 
the trigeminus (Vallin and Demange). In this way the patient 
may lose all his teeth in a few months. It is very interesting 
to note that this may be connected with laryngeal crises, a fact 
which would indicate that there may be some truth in the view 
advanced by Buzzard (British Med. Journal, February 19, 
1886), according to which the centre for bone nutrition lies 
quite close to that of the vagus. 

The so-called mal perforant dn pied (perforating ulcer), which 
begins with the formation of a bleb and leads to abscess forma- 
tion and necrosis of the tendinous and bony portions of the 
feet, is due to some trophic disturbance, and may become a 
source of great discomfort to the patient. 

Affections of the bones and joints, which are also of trophic 
origin, belong to the more frequent complications of tabes. 





Fig. 174. Fig. 175. 

Fig. 174. — Erosion of the Head of the Humerus in Tabes Dorsalis. Fig. 175.— 
Normal Humerus. (After Charcot.) 

The bones become extraordinarily brittle and fractures fre- 
quently occur without pain, and one could almost say without 
the knowledge of the patient. The seat of such fractures is 
most commonly in the femur, and, more especially in old 



TABES DORSALIS. 



657 



women, in the neck 
of that bone. This 
remarkable fragility 
is of especial mo- 
ment when it occurs, 
as it sometimes does, 
in the bones of the 
spinal column, and 
particularly in its 
lumbar portion, and 
gives rise to spondy- 
lolisthesis without it 
being possible to de- 
cide whether or not 
the cartilages and 
ligaments were first 
affected and the dis- 
ease of the bones 
Avas only secondary 
(Kroenig, Zeitschr. 
f. klin. Med., 1888, 
xiv, 1, 2). 

Among the joint 
affections which are 
not essentially differ- 
ent from those pro- 
duced by arthritis 
deformans, the " ar- 
thropathie dcs ataxi- 
ques " or " Charcot's 
joint," because it was 
first described by 
him, deserves par- 
ticular mention. Ac- 
cording to his de- 
scription, there de- 
velops in the course 
of one night, with- 
out any appreciable 
cause and without 
pain or febrile move- 
ment, a swelling: of a 
42 




Fig. 176.— Skeleton of 
cot. ) (The original 



2-mmr 1 

a Tabetic Foot. (After Char- 
the pathological museum of 



is in 

Charcot's department in the Salpetriere in Paris.) 1-5, 
metatarsal bones. 6, internal cuneiform bone. 7, mid- 
dle cuneiform bone. 8, fragment of the external cunei- 
form bone. 9, cuboid bone. 10 and ri, fragments of 
the scaphoid bone. 12 and 13, the astragalus. 14, the 
os calcaneum. 



658 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

joint — for instance, the knee, shoulder, elbow, or hip. In the 
course of a few days there is noted a collection of fluid in the 
joint and in the periarticular bursas, and on puncture a lemon- 
yellowish transparent serum can be withdrawn. In one or two 
weeks later one is able to make out more or less well-marked 
crepitation, due to changes in the joint surfaces. The joint 
becomes extraordinarily movable, and luxations frequently 
occur, especially when the ends of the bone are worn away 
(Figs. 174 and 175). 

Occasionally the tarsus is affected by the process. In such 
cases a marked swelling of the foot occurs in a relatively short 
time, the joints become affected in the way stated above, and 
at the post-mortem examination the tarsal bones are found to 
be altered in the manner represented in Fig. 176 ("tabetic 
foot"). 

The real cause of the affection is not yet known. While 
Charcot considered it due to an atrophy of the anterior gan- 
glionic cells in the cord, Virchow pointed out that it might be 
due to a state of lowered nutrition of the bone following a 
disturbance of nerve influence. Oppenheim and Siemerling 
demonstrated a degeneration in the peripheral nerves, and ac- 
cording to Volkmann the analgesia produced by tabes creates 
a predisposition to the occurrence of the joint affection which 
he attributes to disturbances in the cartilages. Rotter divides 
the cases into three groups — true arthritides deformantes, 
primary fractures of the joints, and a third class in which there 
are most pronounced changes, but in which we are unable to 
determine whether they are due to an arthritis or a primary 
fracture. 

We may add that arthrectomy has lately been performed 
several times for tabetic affections of the knee joint, and has 
been followed by success (Wolff, Sitzung der Berliner med. 
Gesellsch., 7. Marz, 1888, Deutsche Med.-Ztg., 1888, 22, p. 268). 



LITERATURE. 

3. Spinal Cord. 

c. Vasomotor and Trophic Changes. 

Portalier. These de Paris, 1884. (Trophic Changes in the Pre- ataxic Stage 

of Tabes.) 
Rossolymmo. Arch. f. Psych, u. Nervenkh., 1884, xv, 3. (Trophic Changes in 

the Skin.) 



TABES DORSALIS. 



659 



Janovvsky. Wiener med. Presse, 1885, xxvi, 8. (On Exanthematous Eruptions 

in Tabetics.) 
Hoffmann. Berliner klin. Wochenschr., 1885, xxii, 12. 
Browne and d'Arcy Power. St. Bartholomew's Hospital Reports, 1886, xviii. 

(" Mai perforant du pied.") 
Galippe. Gaz. des hop., 1886, 58. (Affections of the Teeth.) 
Krbnig. Wirbelerkrankungen der Tabiker. Deutsche Med.-Ztg., 1886, vii, 

101. 
Hinze. Das Plantargeschwiir und sein Verhaltniss zur Tabes. St. Petersburger 

med. Wochenschr., N. F., 1886, iii, 26-28. 
Menetrier. Annal. de dermat. et syph., 1886, vii, 1. (Mai Perforant affecting 

the Hands.) 
Molliere. Lyon med., 1887, lv, p. 377. (Onset of Tabes with Trophic 

Changes.) 
Suckling. Brit. Med. Journ., April 6, 1885. (Perforating Ulcer as the First 

Symptom of Tabes.) 
Marshall, J. G. Lancet, January, 1885, i, 1. 
Joffroy. Gaz. des hop., 1885, 133. (Tabetic Foot.) 

Richardiere. Revue de med., Fevr. 3, 1886, vi. (Arthropathy in the Finger- 
joints.) 
Anderson, J. Wallace. Brain, 1886, xxxiv, p. 224. (Arthropathy.) 
Kosinzow. Med. Observ., 1886, 17. 

Lowenfeld. Munch, med. Wochenschr., 1887, xxxiv, 20. (Arthropathy.) 
Rotter. Arch. f. klin. Chir., 1887, xxxvi, 1. (Arthropathy of Tabes.) 
Kramer. Prager med. Wochenschr., 1887, xii, 33. (Tabetic Foot.) 
Kahklen. Virchow's Archiv, 1887, cix, 2. (Arthropathy.) 
Porter. New York Med. Record, October, 1887, xxxii, 18. (Arthropathy.) 
Dana. Boston Med. and Surg. Journ., October 17, 1887, cxvii. (Arthropathy.) 
Paolides. Des arthropathies tabetiques du pied. Nouv. Iconogr. de la Salpetr., 

1888, 4, 5. 
Collier and Pitt. Transactions of the Pathol. Society, 1888, xxxix, p. 22. 

(Charcot's Joint in the Knee.) 
Kredel. Die Arthropathien und Spontanfracturen bei Tabes. Volkmann's 

Samml. klin. Vortr., 1888, 309, Chir. Nr. 
Dejerine. Sur l'atrophie musculaire des ataxiques. Paris, 1889. 
Suckling. Brit. Med. Journ., 1889, p. 1009. (Muscular Atrophy.) 
Verneuil. Bull, med., 1890, 76. (Pathological Fractures.) 
Konig. Progr. med., 1891, 44. 
Rosin. Zur Lehre von den trophischen Kiefererkrankungen bei Tabes. Deutsche 

Zeitschr. f. Nervenkh., 1891, 5, 6. 
Sterne. Arthropathia tabidorum. Inaug.-Dissert., Berlin, 1891. 
Goldscheider. Atrophische Lahmung bei Tabes. Zeitschr. f. klin. Med., 

1891, 5, 6. 
Grosse. Ueber Muskelatrophie bei Tabes. Inaug.-Dissert., Berlin, 1892. 
Lipkau. Atrophische Lahmungen bei Tabes. Inaug.-Dissert., Berlin, 1892. 

For the last few years only we have known that the periph- 
eral nerves play a large and important part in tabes ; previous 
to this Turck and later Friedreich had reported alterations 



660 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

in the mixed nerves, but we were ignorant of their character 
until the publication of the work of Westphal (1878), which was 
soon followed by other articles, among them those of Dejerine, 
Pitres and Vaillard, Oppenheim and Siemerling, Sakaky and 
Pierret. The results of their work showed that the peripheral 
nerves suffer a parenchymatous degeneration, a destructive 
process, which, being associated with an increase of the peri- 
neurium, a proliferation of the nuclei, and extensive connective- 
tissue formation, leads to a final atrophy of the nerve elements. 
This peripheral neuritis is not necessarily followed by marked 
symptoms, but, according to our views, it is the main factor in 
the production of the analgesias which are often observed so 
early in the course of tabes, and to which O. Berger has already 
directed attention. Under certain circumstances this neuritis 
may produce deformities ; thus if it involve the nerves which 
supply the muscles of the plantar surface of the foot these 
latter atrophy. The muscles concerned are those of the inner 
surface of the foot affecting the great toe, those of the outer 
surface to the little toe, the flexor brevis communis, and the 
interossei ; the plantar aponeurosis retracts, and the toes be- 
come flexed and immovable (Fig. 177). 

If larger nerves be affected by the process, the symptoms, 
which are characteristic of neuritis, and which have been de- 
scribed on page 386, make their appearance. They are chiefly 
pains, motor disturbances, and muscular atrophies. To this 
class belong the musculo-spiral paralyses caused by tabes, de- 
scribed by Strumpell (Berl. klin. Wochenschr., 1886, xxiii, 37), 
lesions of the median, described by Remak (ibid., 1887, xxiv, 
26), and lastly, lesions of the peroneus longus, as described by 
Joffroy (Gaz. hebdom., 1883, xxxii, 48). Lately Dejerine has 
described a widespread muscular atrophy in tabetics, which 
has its origin in a peripheral neuritis (Neurite motrice peri- 
pherique des ataxiques, Revue de med., 1889, 2). The obser- 
vations of Remak seem to indicate that the muscles which 
are subjected to an unusual strain in the patient's occupation 
are particularly prone to become atrophied. In confirmation 
of this I can add two cases of my own: (1) In a cigar-maker, 
who exerted particularly the first three fingers of the right 
hand in making the cigar-tips, atrophy developed in the muscles 
of the ball of the thumb supplied by the median. (2) A den- 
tist with tabes, who overexerted the musculature of the hand in 
filling teeth and in other manipulations, came under my obser- 



TABES DORS A LIS. 66 1 

vation on account of an atrophy of the hypothenar muscles 
supplied by the ulnar. Similar cases are not uncommon. 




Fig. 177.— Plantar Flexion of the Toes in the Course of Tabes 

(personal observation). 

It is not at all rare in the course of tabes for the peripheral 
nerves to be attacked by neuralgias ; the sciatic nerve calls for 
first mention, as it is usually affected early in the disease and 
very severely. We have already stated on page 372 that 
double sciatica is more particularly a frequent accompaniment 
of tabes. Branches of the pudic nerve may also be affected, 
and often recto-vesical neuralgia may be a source of great 
trouble (Neftel, Arch. f. Psych, und Nervenkrankheiten, 1880, 
10); in this the patients complain of a painful burning sensa- 
tion in the rectum after each defecation, which is often fol- 
lowed by marked depression of spirits, and the longer the in- 
terval between the acts of defecation and the firmer the con- 
sistence of the stool, the more intense becomes the suffering. 
After all, it is not easy to distinguish the peripheral from the 
above-described central affection, which may run a similar 
course. 

LITERATURE. 

4. The Peripheral Nerves. 

Sakaky. Arch. f. Psych, und Nervenkh., 1884, xv, 2. (Degeneration of the 
Peripheral Nerves.) 

Stern, Bolko. Ibid., xvii, 3. (Anomalies of Sensation.) 

Oppenheim und Siemerling. Arch. f. Psych, u. Nervenkh., 1887, xviii, 2. (Af- 
fections of the Peripheral Nerves.) 



662 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Dejerine. Gaz. de Paris, 1888, 10, 11, 12. 

Dejerine. Revue de med., 1889, ix, 2, 3, 4. 

Dejerine et Sollier. Arch, de med. experiment., 1889, i, 2. 

Dejerine. Radialislahmung bei Tabes. Deutsche Med.-Ztg., 1890, 20, p. 231. 

Biernacki. Analgesie des Ulnarisstammes. Neurol. Centralbl., 1894, 7. 

We will now attempt to say something- as to the relative 
frequency of the symptoms and the time of their occurrence, 
but, of course, such statements can not lay claim to accuracy, 
and can only serve to give an approximate idea concerning the 
points in question. 

Among the most frequent symptoms belonging to the brain, 
are, as we have already shown, lesions of the cranial nerves, 
and particularly of the oculomotorius, by which transient diplo- 
pia and irregularities in the condition of the pupils (anisocoria, 
myosis) are produced ; next come lesions of the abducens. 
Almost as frequently will one recognize disturbances of the 
vagus, among which the gastric crises deserve particular men- 
tion. Among the spinal symptoms belonging to this category 
the first to be mentioned are the manifold disturbance of sensi- 
bility, among them cutaneous analgesias, particularly in the 
lower extremities, then, the paraesthesias and the lancinating 
pains which occur more particularly in the legs ; how far these 
symptoms in a given case are due to disease (irritation) of the 
posterior spinal roots or to lesions of the peripheral nerves can 
only be determined by microscopic examination. At all events, 
degeneration of the peripheral nerves in the most varied cuta- 
neous areas is to be classed among the regular occurrences in 
tabes. The disappearance of the patellar reflex and some form 
of the various bladder troubles are almost constant accompani- 
ments of the affection, and these, taken in connection with the 
symptoms just mentioned, must be considered as the founda- 
tion for the diagnosis. 

Lesions, particularly atrophy, of the optic nerve, symptoms 
of irritation and paralysis in the domain of the fifth nerve, and 
ataxia of the lower extremities, are frequent but less regular 
occurrences. 

Less frequently met with are the laryngeal crises, due to 
lesions of the vagus, and affections of the nerves of taste and 
of the accessorius ; the same may be said of the psychoses, 
hemiplegias, and attacks of epilepsy observed in the course of 
tabes. Certain disturbances of sensibility, the so-called rectal 
crises, cutaneous hyperaesthesias, neuralgias of the peripheral 



TABES DORSALIS, 



663 



spinal nerves, paraplegia of the legs, the tremor, and disturb- 
ances in the sexual functions also belong to this category. The 
trophic disturbances, the muscular atrophy, the falling out of 
the nails and hair, the " mal perforant du pied" and Charcot's 
disease of the joints are also comparatively rare. 

To the symptoms which occur only seldom, one might 
almost say exceptionally, belong those referable to the hypo- 
glossal, the auditory, and the facial nerves ; among the motor 
disturbances of spinal origin, the so-called associated move- 
ments and ataxia of the upper extremities, among the sensory 
disturbances, the so-called polyassthesias, double sensations, and 
delayed sensations belong to this class ; marked diminution in 
the muscular strength is also exceptional. 

As to the time at which these various symptoms severally 
arise, it is even more difficult to give reliable data, since there 
exists no uniformity ; still, one can state with some amount of 
certainty that next to a feeling of slight weariness, particularly 
in the legs, the lesions of the oculo-motor and abducens are 
often the first to make their appearance ; the disturbances of 
sensibility, particularly analgesia and paresthesia, as a rule 
also occur early, while lancinating pains make their appear- 
ance at a later period. The gastric crises are observed rela- 
tively early, and bladder troubles are among the more frequent 
occurrences before the disease has advanced very far. The 
disappearance of the patellar reflex, as it usually constitutes 
one of the initial symptoms of the disease, plays an important 
part in the diagnosis, as we have already shown. Pronounced 
motor disturbances, particularly ataxia of the lower extremi- 
ties, are often not observed until later in the disease, often only 
after years ; and paraplegia of the legs, when it occurs at all, 
characterizes the last stages of the disease. Optic atrophy 
sometimes makes its appearance relatively early ; in other in- 
stances it occurs only at a late period and comes on very grad- 
ually. For the time of its occurrence no definite rules can be 
laid down. Hemiplegias, epileptic attacks, and psychical dis- 
turbances, if they occur at all, manifest themselves sometimes 
earlier, sometimes later. As far as our own observations go, 
the trophic disturbances mentioned above, particularly the 
muscular atrophies and Charcot's joint affection, usually belong 
to the later stages. 

The course of tabes is rarely markedly influenced by com- 
plications, but such may nevertheless occur. Lesions of the 



664 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

pyramidal tracts in the spinal cord (Eulenburg, Deutsche med. 
Wochenschr., 1887, 35), valvular diseases of the heart, espe- 
cially aortic insufficiency, Graves* disease, pernicious anaemia, 
diabetes, general paralysis, and bulbar paralysis are to be re- 
garded as complications. Coexisting hysterical symptoms we 
may at times not be able to distinguish from those arising from 
the tabetic changes. 

LITERATURE. 
j. Complications. 

Oppenheim. Berliner klin. Wochenschr., 1884, xxi, 38. (Hemicrania and 

Tabes.) 
Leichtenstern. Deutsche med. Wochenschr., 1884, x, 52. (Pernicious Anaemia 

and Tabes.) 
Oppenheim. Berliner klin. Wochenschr., 1885, xxii, 49. (Diabates complicat- 
ing Tabes.) 
Reumont. Ibid., 1885, xxiii, 13. (Diabetes with Tabes.) 
Grasset. Arch, de Neurol., Juillet, 1886, xii. 
Fischer. Centralbl. f. Nervenkh. u. Psychiatr., 1886, ix, 18. (Diabetes with 

Tabes.) 
Leyden. Centralbl. f. klin. Med., 1887, viii, 1. (Cardiac Affections with 

Tabes.) 
Eulenburg. Deutsche med. Wochenschr., xiii, 35. (Tabes combined with 

Motor System Disease of the Spinal Cord.) 
Groedel. Deutsche med. Wochenschr., 1888, xiv, 25. (Cardiac Affections 

with Tabes.) 
Stransky. Prager med. Wochenschr., 1888, xiii, 25. (Tabes with Muscular 

Atrophy.) 
Lichtheim. Deutsche Med.-Ztg., 1890, 16, p. 187. (Tabes and Pernicious 

Anaemja.) 
Jolly. Munch, med. Wochenschr., 1891, 23, p. 406. (Tabes with Hemiatrophia 

Faciei.) 
Kuh, Sidney. Arch. f. Psychiat. u. Nervenkh., 1891, xxii, 3. (Complication with 

Meningitis Cerebro-spinalis.) 
Souchay. Tabes mit Herzaffectionen complicirt. Charite-Annalen, 1893, xv i n - 
Marie et Marinesco. Revue neurol., 1893, 10. (Tabes with Graves' Disease.) 

Course. — About the general course of the disease the fol- 
lowing remarks will hold good in a large number of cases : A 
middle-aged person who has become infected with syphilis 
some years previously, usually from eight to fifteen years be- 
fore, begins to complain of slight fatigue on walking and occa- 
sional pains in the lower extremities. In spite of all treatment 
the pains continue to be troublesome, and occasionally become 
so severe that they disturb the patient's rest at night or even 



TABES DORSALIS. 665 

render sleep impossible. At the same time it appears to him 
that his vision is becoming affected, and he complains particu- 
larly that he sees double, and in consequence suffers from ver- 
tigo. The diplopia may last only for a few moments at a time. 
The vertigo, which at the onset of the trouble was insignifi- 
cant, becomes more and more pronounced, more especially in 
the dark, so much so that it is almost impossible for the patient 
to pass through a dark room without help. He also discovers 
that he staggers or falls to one side in the morning when in 
washing he covers his face with the towel, and only regains his 
equilibrium when his eyes are free again. Only rarely do dis- 
orders in the innervation of the larynx occur in the incipient 
stage of the affection, but an abductor paralysis may be found 
very early (Grabower, Deutsche med. Wochenschr., 1893, 18), 
and the laryngeal examination should therefore never be 
omitted in suspicious cases. Finally, he complains that he is 
obliged to pass water more often than usual, and that he con- 
sumes more time and must exert himself more when urinating 
than previously. The objective examination shows that there 
is widespread anaesthesia, particularly analgesic areas, about 
the lower extremities, and a loss of the patellar reflex. He 
may be inconvenienced in this way for years without his con- 
dition becoming serious. He suffers more or less all the time, 
sometimes quite severely, but, on the whole, his existence is 
quite bearable. The state of his mind is hopeful, for the daily 
occupation has not yet been interfered with by the disease. 

The aspect of affairs is quite different when the patient suf- 
fers from gastric disturbances. The appetite becomes poor, 
and occasionally — sometimes for weeks at a time — there is morn- 
ing vomiting, which is quite profuse and occurs as soon as the 
patient awakes, when, without effort, watery, slimy masses are 
discharged. After lasting for a longer or shorter time this 
ceases, probably only to return later on. The appearance of 
the patient, which was previously natural, now becomes al- 
tered for the worse. The skin becomes yellow and wrinkled, 
and his friends and acquaintances, w T ho have not seen him for 
some time, begin to inquire about his health. At the same time 
a new symptom makes its appearance, and he notices that his 
gait is becoming uncertain and that in walking he must invoke 
his eyes to aid his legs, which, instead of carrying out the 
movements he intends, are thrown out in a peculiar aimless 
manner, so that if he be not led or supported he runs the risk of 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 

tumbling down. This trouble in walking, which is associated, 
perhaps, with occasional gastric and more rarely with laryn- 
geal crises, may likewise continue for years ; but if the ataxia 
implicates the upper extremities, as happens in a small propor- 
tion of the cases, it may so interfere with the patient's occupa- 
tion that he may be unable to continue it. In the meanwhile 
the bladder symptoms become more prominent and are aggra- 
vated to such an extent that it becomes necessary for the 
patient to wear some sort of receptacle, while the marked con- 
traction or inequality of the pupils is apparent even to the 
layman. 

Gradually another change for the worse in the gait comes 
on. The legs, which, although thrown out in the characteristic 
manner, in other respects performed their duty and even en- 
abled the patient to cover considerable distances, begin to be 
fatigued on the slightest exertion ; they become heavier and 
heavier, and it becomes more and more difficult, and at last 
needs the greatest effort, to walk at all. The legs are so weak 
that they are no longer able to support their owner, who is 
forced to take to the invalid's chair, and in this he ends an 
existence, the last years of which are as wretched as could be 
imagined, especially if atrophy of the optic nerve has robbed 
him of sight and the lancinating pains make his days and nights 
miserable. When the disease progresses in this or a similar 
manner its duration varies from ten, fifteen, even to twenty 
years or more. It can, however, be considerably shorter. I 
have seen cases in which only from three to five months 
elapsed between the beginning of the affection, from the first 
appearance of the disturbances in the movements of conver- 
gence of the eyes, to the appearance of well-marked paralysis 
of the legs. 

On the other hand, there are cases in which the course may 
extend over a space of thirty or more years ; in these paralytic 
symptoms may not come on at all, and the ataxia may continue 
to the end. There are tabetics who during their entire illness 
are hardly prevented at all from carrying on their work ; they 
are always able to be up and about, and it appears as if the 
different symptoms never attained their full development. 
These are the so-called " formes frustes" of the French, analo- 
gous to those with which we have already become acquainted 
in Graves' disease and in multiple sclerosis. Again, in other 
cases, tabes sets in with brusque symptoms, such as apoplecti- 



TABES DORSALIS. 



66? 



form attacks, disturbances of speech, and lesions of the optic 
nerve, and then pursues a mild course for a long period — vio- 
lent symptoms, such as laryngeal crises, intense neuralgias, 
etc., only occurring occasionally ; these are the so-called atyp- 
ical forms of the authors. 

From what has been said it is evident how difficult it is 
to make a positive statement concerning the general course of 
the affection. Scarcely one case follows the same course as 
another, and it often requires a great amount of caution and 
experience to enable one to take a correct view of all that 
occurs. 

Just as much uncertainty exists about the prognosis, which 
is influenced by various factors. One most important question 
is, of how long standing is the disease, for in recent cases in 
which there are no other symptoms than disturbances of sen- 
sibility and absence of the knee jerks, and the course of which 
has not as yet exceeded three or four months, and in which 
there is no ataxia, the prognosis is not at all unfavorable, and 
the disease is under some conditions curable. Advanced cases 
of tabes in which there are numerous spinal and cerebral symp- 
toms offer a much more serious prognosis, but even here the 
possibility of cure is not excluded, though the highest percent- 
age of recoveries is estimated at one per cent (Eulenburg). Of 
course, one can not expect that the anatomical changes will 
disappear, and at the autopsy a widespread degeneration of 
the posterior columns has been found in cases in which during 
life all symptoms had practically disappeared. In the majority 
of the so-called recoveries from tabes one is led to believe that 
there was a mistake in the diagnosis, and that these were cases 
of chronic nicotine poisoning, peripheral neuritis, hysteria, 
neurasthenia, etc. The prognosis of old cases with paraplegia 
of the legs, paralysis of the bladder, and so forth, is altogether 
unfavorable, and any attempts at cure are not only useless, but 
may even interfere with the comfort of the patient. 

It is a matter of indifference, so far as the prognosis is con- 
cerned, whether one is able to demonstrate that the patient has 
at one time or other been infected with syphilis or not ; a so- 
called specific or luetic tabes, especially when the infection has 
taken place ten or twenty years previously, does not afford a 
better outlook than the more rare idiopathic affection. 

It is clear, then, that one must be very cautious in predict- 
ing the duration of the disease ; one can not say definitely how 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 

many years a tabetic patient has to live, and just as little should 
one attempt to make a positive statement as to how long the 
patient will be able to work. The condition may remain quite 
endurable for months or even years, and the outlook may ap- 
pear quite hopeful, particularly in regard to the capacity for 
work, and yet suddenly a marked change may take place ; pro- 
nounced ataxia, cerebral symptoms, or the like may manifest 
themselves, which render the patient incapable of following 
any occupation. The more cases one sees, the more cautious 
does one become in giving a prognosis, and the more distrust- 
ful of the reports of so-called cures — at least when old cases are 
concerned. 

Diagnosis. — As one can readily see from what has been 
said, the diagnosis of tabes is sometimes one of the simplest 
possible tasks for the physician ; in other instances it can not 
be made with certainty for a long time. Thus it may under 
certain circumstances be very difficult to differentiate between 
the disease under consideration and complicated cases of syph- 
ilis of the brain and spinal cord, diabetes, or hysteria. It seems 
perfectly possible for one to consider a severe case of neuras- 
thenia for a long time as one of tabes, but the further course 
and final success of therapeutic measures will demonstrate the 
error. When in the course of tabes the sensory and bladder 
disturbances are only slightly marked, there may be question 
of the existence of a chronic anterior poliomyelitis, but usually 
the lancinating pains, the paraesthesias, the affection of the eye 
muscles, and the mere fact that bladder symptoms exist at all, 
afford sufficient grounds on which to base a diagnosis. In dis- 
eases of the vertebral column, in the course of which lancinat- 
ing pains, " Westphal's sign," and bladder symptoms may be 
found, an examination will reveal that the vertebral column 
itself is affected, and the spinous processes are painful on pres- 
sure — a condition which is sufficient to settle the diagnosis. 
The mistake of considering a tabophobe, or a person who im- 
agines he has tabes, as a real tabetic, can only occur when a 
careful examination is neglected, and the physician is afraid to 
adopt any energetic, psychical as well as somatic, treatment. 
As soon as this is instituted the tabetic symptoms will turn out 
to be mere hypochondriacal notions, and recovery will quickly 
follow. 

It is of practical importance to note that the various symp- 
toms occurring in tabes are also observed in other affections. 



TABES DORSALIS. 



669 



In these cases there is much room for errors in diagnosis, the 
most important of which we wish to bring to the reader's 
attention. 

Paralysis of the eye muscles and pupillary symptoms are, 
as we have remarked, very common in the course of tabes, 
Another affection in which they also occur is multiple sclerosis. 
Here, however, diplopia as well as strabismus are rare, while, 
on the other hand, nystagmus is very frequent, and the pupil- 
lary reaction to light is preserved. Myosis occurs in this affec- 
tion as well as in tabes, but whereas in the former the pupils 
contract still more under the influence of light, in tabes they 
usually remain immobile under the same circumstances. 

Symptoms referable to the optic nerve — amblyopia, for in- 
stance — are also observed as the effect of different poisons (page 
39). In such cases the history will be of great assistance to 
us in making the diagnosis. Amblyopia developing in the 
course of multiple sclerosis is not accompanied, as in tabes, by 
a contraction of the field of vision, nor does it steadily grow 
worse ; but remissions occur, and the improvement may even 
last for a considerable time. It has already been shown on 
page 620 that the optic atrophy of multiple sclerosis differs in 
important points from that occurring in tabes. It should also 
be remembered that there is an optic atrophy in which the 
morbid process is confined to the optic nerve, and in which it 
is impossible to demonstrate any general nervous disease. 

The various visceral " crises," in which tabes abounds, can 
likewise be produced by independent affections of the vagus. 
Here one must rely upon the more characteristic symptoms of 
tabes, particularly Westphal's and Romberg's signs. That 
" gastric crises " alone can not enable one to make the diag- 
nosis is all the more to be insisted upon since Debove has 
observed them in neurasthenics (Soc. des hop., seance 1888, 
xii, 28). 

The motor disturbances which we find here, and of which 
the most important is the ataxia of the lower extremities, ap- 
pear not only in the course of tabes, but also in other diseases 
in which one is unable sometimes to ascertain their anatomical 
basis. This is more especially true of the so-called functional 
ataxias (Gallard, Jaccoud), which develop sometimes with, 
sometimes without, sensory disturbances, and are associated 
with no other symptoms. Ataxia has likewise been observed 
developing slowly or quickly after diphtheria (Berl. klin. 



6yo DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Wochenschr., 1887, 49, p. 930), after quickly succeeding preg- 
nancies, and in the course of diabetes ; and the question must 
remain undecided whether it is to be considered as the expres- 
sion of a severe general affection, of a faulty composition of 
the blood and an imperfect innervation dependent upon it, or 
as the result of a peripheral neuritis developing under the 
influence of an infectious agent. However, it can not be diffi- 
cult in a given case to determine whether the ataxia is to be 
regarded as of spinal or tabetic, or as of functional or of in- 
fectious origin. In cases of hysteria the differential diagnosis, 
as has been already pointed out, may present very great diffi- 
culties (Pseudo-tabes hysterique, Gaz. med. de Paris, Septembre 
20, 1890). 

The lancinating pains occur also in affections of the verte- 
bral column, e. g., in Pott's disease, when the posterior roots 
are irritated, but the deformity and the tenderness of the ver- 
tebras upon pressure will make the diagnosis clear. 

Other pains, following the course of various larger nerves, 
which can last for weeks or months without marked exacerba- 
tions, and be accompanied by paraesthesias, formication, numb- 
ness, etc., are observed not only in tabes, but also in peripheral 
neuritis, following, for example, the abuse of alcohol. If to 
these a temporary loss of the patellar reflex be added, we have 
the picture of what is called pseudo-tabes, and a cautious and 
often-repeated examination is necessary in order to make the 
differential diagnosis. The history and the further course of 
the disease, which in alcoholic neuritis may become favorable 
after the removal of the cause, should always be taken into 
consideration (Higier, Deutsche med. Wochenschr., 1891,34; 
Fournier, Miinchener med. Wochenschr., 1892, 10). 

We have already pointed out on page 650 the circumstances 
under which Westphal's sign may be present, and we can not 
insist too strongly that it is an error, or at least a too hasty 
conclusion, to think only of tabes whenever the patellar reflex 
is absent. On the other hand, we must not imagine that its 
presence puts tabes out of the question, for the possibility of 
the existence of this disease is not at all excluded when the 
reflex is found to be normal. 

Pathological Anatomy. — Considered from the pathological 
standpoint, tabes represents a degenerative process in which 
the entire nervous system takes part. The reason that we have 
been unable to demonstrate in all cases the participation of all 



TABES DORSALIS. 



671 



the nerves — that in many cases, for example, the cord seems to 
be the part most involved while the brain and its nerves appear 
less affected — lies in the fact that we have been accustomed to 
examine the cord with the greatest accuracy, while the brain 
and peripheral nerves were considered only of secondary im- 
portance ; and, secondly, in the fact that many cases are ter- 
minated by intercurrent diseases before the degenerative 
process has had time to develop in all directions. 

This degeneration, which consists principally in the death 
of the nerve elements and an increase of the supporting tissue 
(Leyden), presupposes a certain change in the nervous system, 
the nature of which we do not as yet know, and which is 
peculiar to the individual either as the result of hereditary 
influences or which has been acquired later through syphilitic 
infection. The congenital predisposition is not sufficient to 
produce an outbreak of the disease. For this some one of cer- 
tain exciting causes, of which we shall speak later, is needed. 
On the other hand, the changes produced in the nervous sys- 
tem by a syphilitic infection are able of themselves to lead to 
the production of tabes. As to the manner in which heredity 
works in the production of these changes, we are not in a posi- 
tion even to hazard a conjecture, nor are we by any means cer- 
tain of the precise mode of action of syphilis. In this latter 
case, however, it is, according to our idea, most probable that 
the changes are a result of a syphilitic affection of the blood- 
vessels. It is, in our opinion, less likely that a poison (" toxine "), 
which affects the nervous system, is developed secondarily, in 
which case tabes would have to be regarded as a post-syphilitic 
affection, just as paralysis of the soft palate is a post-diphthe- 
ritic affection (Striimpell) ; and it would be still harder to im- 
agine that the syphilitic virus becomes localized in the nervous 
system, and, as such, later produces the disease (Rumpf). One 
could in the last case not help but ask how it is possible for 
ten, fifteen, or more years to elapse between the syphilitic 
infection and the appearance of the first tabetic symptoms, a 
circumstance which, on the other hand, could be easily ex- 
plained by assuming the existence of anatomical changes which 
are due to a diminution in the blood supply and require a rela- 
tively long time for their development. 

The degeneration begins probably always in the peripheral 
nerves. The terminations of the cutaneous sensory nerves may 
be the first to become affected. The admirable researches of 



672 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

Dejerine, Oppenheim, Siemerling, and others, have clearly 
demonstrated the participation of the peripheral nerves in the 
tabetic process, and there is no doubt but that they appear just 
as much degenerated as the posterior roots, in which the atro- 
phy was shown to be most marked between the spinal ganglia 
and the cord, while the peripheral portion was often relatively 
quite free (Dejerine, Compt. rend, de la Soc. de biol., 1882, p. 
215). The degree to which the several cutaneous nerves are 
attacked varies. Those of the legs are usually more affected 
than those of the upper extremities. No definite rule can be 
said to exist. Sometimes, and this often happens, the periph- 
eral ends of certain of the cranial nerves are the first to be- 
come diseased — e. g., those of the optic, the oculo-motor, and 
the abducens — and then the symptoms described above appear 
in the initial stage. At any rate, the first symptoms develop 
in consequence of lesions of peripheral end organs. 

The degenerative process in the cord, which occurs later, 
is the most prominent pathological feature at the autopsy, and 
formerly was considered the only, or at least the only charac- 
teristic, lesion, This explains why tabes was and is still con- 
sidered, by the majority of authors, as a disease of the spinal 
cord. According to our idea this is not true. It is rather an 
affection of the entire nervous system, in which the cord is 
not even the first part to become affected, but later is altered 
in such a characteristic and striking manner that we can not 
be surprised if the other, less marked, conditions were over- 
looked. Though the changes in the cord have long been rec- 
ognized, the views as to their origin are still conflicting and 
the most varied interpretations have been put forward. We 
do not care to enter into an account of the controversies, but 
will only bring before our readers succintly the conclusions 
arrived at as to the nature of the affection. It consists prob- 
ably of a primary degenerative atrophy of the nerve fibres, 
which is followed by a secondary increase of the supporting 
tissue. As the degeneration takes place slowly, few compound 
granular corpuscles are found, and only in older cases can cor- 
pora amylacea be demonstrated. The grayish discoloration of 
the posterior columns depends upon the destruction of the 
medullary sheaths. A marked degree of atrophy is to be no- 
ticed in the posterior columns, and in advanced cases the 
entire cord appears narrower and thinner than is normal. 
On cross-section it is readily demonstrable that besides the 



TABES DORSALIS. 



673 



posterior columns the posterior gray horns and the posterior 
roots also become atrophied. The condition of the posterior 
roots has been carefully studied by Leyden, who noted the 
frequent atrophy in them ; according to his conception, the 
changes in the posterior columns are a result of the changes in 
the posterior roots, so that we have " a progressing affection of 
the sensory portions of the spinal cord." This view is still 
upheld by Leyden, in spite of various objections which have 
been raised against it (Redlich, Marie, and others ; see the latest 
article of Leyden in the Zeitschr. f. klin. Med., 1894, xxv, 1, 2). 
Moreover, it is of interest to note that certain portions of the 
cord seem, as a rule, to be spared, while others are almost 
always involved in the degeneration which affects both sides of 
the cord symmetrically. The lesion is of the character which 
we have learned to recognize in the so-called " combined system 
diseases " — that is, certain systems of fibres which have certain 
anatomical and physiological relations to one another become 
diseased, while others are unaffected. It is also seen that not 
all portions of the posterior columns are 
implicated equally (Striimpell), but that 
the extent of the lesion differs according 
to its situation. For example, it is most 
severe in the lumbar region, in which 
only the anterior part is left intact, the 
middle and posterior portions being de- Fig. 178. 






Fig. 179. Fig. 180. 

Fig. 178.— Section through the Cervical Cord in a Case of Commencing Tabes. 
Fig. 179. — Section through the Lumbar Cord in Tabes. Fig. 180. — Section 
through the Cervical Cord in a Case of Advanced Tabes. (After Strum- 
pell.) 



generated. In the cervical region there are to be distinguished 
four fields on either side, of which, two, Goll's columns and a 
part of Burdach's columns, the so-called lateral root fields (into 
which direct fibres enter from the posterior nerve roots), appear 

43 



674 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

degenerated, while two others, one anterior and lateral, the 
other posterior and external (the posterior outer fields of 
Striimpell), appear normal (Figs. 178, 179, and 180). Such a 
distribution of the lesion is frequently observed, but naturally 
not found in all cases. We have already mentioned that the 
posterior gray matter is involved in the process. Lissauer 
deserves credit for having demonstrated (Arch. f. Psych, und 
Nervenkrankheiten, 1886, xvii, p. 376) that here the affection of 
the fibres in Clarke's columns should be distinguished from 
that of the fine and large root fibres in the posterior horns. 
Physiologically this discovery can not as yet be utilized. 

Of the lesions in the medulla oblongata and the brain, pro- 
duced by tabes, the former affect the cranial nerves at their 
nuclei or in their peripheral course. Of the manifold symp- 
toms produced thereby we have spoken before. On the other 
hand, we may have lesions of the cortex, an implication of 
which in many cases can not be called into question. We also 
said that some of the nuclei, particularly those of the eye mus- 
cles, of the vagus, and of the hypoglossus, are affected more 
often and more severely than others, while, for instance, the 
facial, the auditory, and the glosso-pharyngeal remain as a rule 
intact, a fact for which we have no explanation. According to 
Jendrassik's conception (Deutsches Arch. f. klin. Med., 1888, 
xliii, 6), the brain is the primary seat of the tabetic process, so 
that the sensory disturbances and the ataxia are to be consid- 
ered as of cortical origin, and the degeneration in the posterior 
columns, and perhaps those of the direct cerebellar tracts, as 
secondary processes. Until the cortex has been examined mi- 
croscopically in the initial stages of the affection, and some 
constant changes have been demonstrated in it after death, this 
theory, like all the others, will remain nothing more than a 
bare hypothesis, and can be neither contradicted nor yet 
accepted. Such a pathogenesis, however, is not impossible, 
though it is not difficult to bring forward objections to it. 

Lastly, it should be mentioned that Basso (Ann. univers. di 
med. et chir., June, 1886) considers tabes to be an affection of 
the sympathetic system, under the influence of which the cere- 
brospinal lesions develop. He thinks that the anatomical 
changes in the nervous system are at first caused by functional, 
and later by organic disease of the blood vessels, and holds 
that when taken in time tabes is curable. 



TABES DORS A LIS. 



675 



LITERATURE. 

6. Pathological Anatomy. 

Adamkiewicz. Die anatomischen Veranderungen bei Tabes. Congr. internat. 

de Copenhague, 1886. 
Pick. Arch. f. Psych, u. Nervenkh., 1889, 3. (Anatomical Condition in a Case 

with Absence of one Knee-jerk.) 
Flechsig. 1st die Tabes eine Systemerkrankung ? Neurol. Centralbl., 1889, 2. 
Schmaus. Deutsches Arch.f. klin. Med., 1890, xlvi. (Implication of the Lateral 

Columns.) 
Raymond. Contribution a l'anatomie pathologique du tabes, etc. Revue de 

med., 1891, 1. 
Brazzolo. Sulle localizazioni anatomo-patologiche e sulla patogenesi della tabe 

dorsali. Bull. d. scienze med., April, 1891, Heft 4, p. 187. 
Looft. Virchow's Archiv, 1892, cxxviii, 1. (The Degeneration of the Posterior 

Columns formerly regarded as characteristic of Tabes is also found in 

Lepra Ansesthetica.) 
Wollenberg. Arch. f. Psych, u. Nervenkh., 1892, xxiv, 2. 
Nonne. Ibid., p. 526. 
Krauss, E. Ibid., xxiii, 2, 3. 
Redlich. Wiener Jahrb. f. Psych., 1892, 1, 2. (Condition of the Posterior 

Roots.) 
Leyden. Deutsche Med.-Ztg., 1893, 96, p. 1002. 
Leyden. Neurol. Centralbl., 1894, 1, 2. 
Marie, P. Etude comparative des lesions medullaires dans la paralysie geneVale 

et dans le tabes. Gaz. des hop., Jan v. 16, 1894. 
Leyden. Zeitschr. f. klin. Med., 1894, xxv, 1, 2. 

Etiology. — In speaking- of the astiology of tabes, one must 
constantly distinguish, as is evident from the views expressed 
above, between the non-syphilitic and the syphilitic affection. 
In the first case one should above all take into consideration 
the hereditary conditions in order to comprehend the congen- 
ital predisposition which is necessary for the production of the 
disease. By this we do not mean to class tabes among the 
hereditary diseases in the ordinary sense of the word, for it 
certainly can not come in this category ; on the contrary, we 
are justified in assuming that direct inheritance of it is quite 
rare. By heredity in this connection we mean a general neu- 
ropathic inherited tendency, or, in other words, that in the 
family of the patient all kinds of neuroses, not excluding psy- 
choses, have occurred repeatedly. Not only the parents, but 
also more distant relatives, e. g., aunts, uncles, or grandparents, 
may have suffered with general paralysis, epilepsy, melancho- 
lia, hysteria, migraine, etc., and it is just this heredity which 
in the presence of exciting causes is sufficient to open the 



6y6 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

door to the tabetic process. The labors of Charcot (Arch, 
gener. de med., Sept., 1883) and the comprehensive statistics of 
Ballet and Landouzy (Arch, de neurol., 1886, vii, 20) have 
thrown an interesting light upon this subject, and have bril- 
liantly substantiated the view which Trousseau expressed at an 
earlier period, that tabes was (in the sense of the word as 
expressed above) hereditary. Among the German authors 
Mobius has occupied himself particularly with this subject 
(Allg. Zeitschr. f. Psych., 1883, xl, 1, 2). 

The exciting causes which relatively frequently lead to the 
development of tabes (in those with hereditary tendencies) con- 
sist {a) in exposure to cold and wet, to sudden changes of tem- 
perature, and to prolonged living in damp lodgings ; (a) in 
traumatic influences; (c) in certain factors due to the daily oc- 
cupation, the most important of which is overexertion. The 
opinion that sexual excesses may lead to tabes, which has been 
expressed by various authors, must be given up as without 
proof. 

I have never questioned but that exposure to cold, sudden 
changes of temperature, and, particularly, severe wettings, may 
play an important part in the aetiology of the affection ; still, to 
me the following case was particularly convincing : The pa- 
tient, a general agent for several hail-insurance companies, fifty- 
eight years old, had had syphilis thirty-nine years before, since 
which time he had been perfectly well. In August, 1885, while 
estimating the damage caused by a hailstorm, he was drenched 
to the skin, and was obliged to spend several hours in his wet 
boots. Three months later the first tabetic symptoms made 
their appearance — paresthesia and anaesthesia of the legs, loss 
of the patellar reflexes, etc. ; by Christmas, 1885, he was mark- 
edly ataxic, and in the spring of 1886 he was unable to pursue 
his calling. In the summer of 1886 he suffered with intestinal 
crises and intense lancinating pains, and eighteen months from 
the beginning of the affection he had paraplegia of both legs. 
In the early part of 1887 he died from an intercurrent attack 
of pneumonia. When tabes develops in one well on in the fif- 
ties, there must be some particular cause for it, and in this case 
it was, without doubt, the wetting. Similar cases can easily be 
found if the history be carefully taken. 

The role which traumatic influences play in the production 
of the affection is just as certain. In one of my cases, a gov- 
ernment official of high position, who had been affected with 



TABES DORSALIS. 



677 



syphilis twenty-nine years before, met with an accident on a 
glacier in the summer of 1884. He fell and slid some distance 
on a snow field with great rapidity, but no bones were broken 
and no dislocation occurred. A few months later the first 
tabetic symptoms made their appearance, and now the disease 
is well developed. Again, a fall from a height may be the 
cause (Oppenheim) ; Strauss reports numerous traumatic cases 
(Faits pour servir a l'etude des rapports de traumatisme avec 
le tabes. Arch, de phys., Novembre, 1886). From his com- 
munication it is apparent (1) that years may elapse after the 
accident before the disease makes its appearance, and (2) that 
the traumatism may have an influence in determining the seat 
of the early symptoms, particularly of the lancinating pains, so 
that, for example, after a fracture of the lower part of the left 
leg the pains will first make their appearance at that point, and 
so forth. In an article by Spillman and Parisot (Traumatisme 
peripherique et tabes, Revue de med., 1888, 3) there is a table 
which gives the different forms of injury which have been 
followed by tabes. Of great interest also is a case reported by 
Blocq and Onanoff (Arch, de med. exp. et d'Anat. pathol., p. 
387, 1892) in which there was a combination of tabes and trau- 
matic neurosis. 

I have already pointed out, in my book on diseases of the 
laboring classes, that the occupation is not without importance, 
and more especially overexertion — for instance, at the sewing 
machine — or hard bodily labor in general may be the cause 
of the outbreak of the disease in those who are predisposed to 
it. However, the percentage of such cases is not large. Hof- 
mann gives an instance which may be classed partly with those 
cases in which the occupation, partly with those in which trau- 
matism, is the exciting cause. The patient was a laborer en- 
gaged in cutting tin plates, and in the course of his work his 
body was shaken from six to ten thousand times daily ; under 
the influence of these shocks the disease developed (Arch. f. 
Psych, und Nervenkrankheiten, 1888, xviii, 2, 439). 

Concerning the syphilitic tabes, which has been studied 
with the greatest care by Fournier and Erb, it is an undoubted 
fact that syphilis by itself is usually a sufficient cause for the 
disease, and that no other exciting factor is needed for its de- 
velopment. 

We do not know what percentage of persons who have had 
syphilis become tabetic, but we do know for certain that the 



6; 8 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

great majority of tabetics have had syphilis at some time or 
other — according to Erb, sixty per cent ; according to Fournier, 
ninety per cent. Syphilis is more frequently followed by tabes 
than hereditary and exciting causes put together. Out of three 
hundred and forty-five cases of tabes which I have seen in the 
last few years of my practice, in sixty-six a syphilitic history 
was not obtained, while in the other two hundred and seventy- 
nine cases it was demonstrated with certainty, so that my 
figures, although they do not quite correspond to those of 
Fournier, give eighty per cent. Minor points out in his statis- 
tics (Wyestnik psychiatri i nervipatologii, 1888, vi) that tabes 
is much rarer in Russia among the Jews than among the other 
Russians, which is simply due to the fact that the former are 
less frequently syphilitic. The communication of Nagel also 
deserves consideration. He found in 1,403 cases of tabes 
forty-six per cent of syphilitics, and out of 1,450 other pa- 
tients only nine and one half per cent. The time which 
elapses between the infection and the first appearance of 
tabes varies from a few months to one, two, five, fifteen 
years or more. The severity of the syphilis does not ap- 
pear to stand in any relation to the severity of the tabes ; 
for one can observe very pronounced tabetic symptoms after 
an apparently trivial and quickly healed primary sore, whereas 
sometimes after the most severe type of syphilis the general 
affection of the nervous system only appears in its mildest 
form. 

The influence which age and sex exert in the production of 
the disease can only be considered in the non-specific cases. 
It is, however, only of slight importance ; for, although it is 
true that males are far more frequently affected than females 
(the proportion being seven to two), and although most of the 
patients are middle-aged, these facts can very well be accounted 
for by the nature of the several exciting causes, which make 
it comprehensible why men in the prime of life furnish rela- 
tively the greatest contingent of cases. For the special con- 
ditions under which the disease may occur in childhood, and 
the peculiarities presented by tabes in children, the reader is 
referred to the articles mentioned on page 679. 

Lastly, it must be confessed that in a few cases, which, 
however, form an exceedingly small fraction of the whole num- 
ber, no aetiological factor can be made out — neither hereditary 
predisposition, nor exciting causes, nor syphilitic infection. At 



TABES DORS A LIS. 



679 



present we can only acknowledge our ignorance of their patho- 
genesis. 

LITERATURE. 

7. ^Etiology {Age, Sex, etc.). 

Prince. Journ. of Nerv. and Ment. Diseases, 1889, xiii. (Malaria as Cause.) 
Gombault et Ballet. Arch, de med. experim., etc., 1889, 3. (Onset at the Age 

of Seven.) 
Bernhardt. Neurol. Centralbl., 1890,23. (Work with the Sewing-machine.) 
Klemperer. Anhang von Frerichs und Leyden, 1890, xviii, 2. 
Tick. Zur Lehrevon den Tabesformen im Kindesalter. Zeitschr. f. Heilkunde, 

1891, xii. 
Hildebrandt. Ueber Tabes in den Kinderjahren. Inaug.-Dissert., Berlin, 1892. 
Erb. Die Aetiologie der Tabes. Yolkmann's Sam ml. klin. Vortr., 1892, 53. 
Friedrichsen. Tabes bein weiblichen Geschlecht. Inaug.-Dissert., Berlin, 

1893. 

Prognosis. — From what has been said, we may infer what 
the prognosis will be. Though it is not absolutely unfavorable 
quoad vitam, inasmuch as the patient may linger on for years, 
and sometimes tens of years, one should not forget that in 
general the course is unfavorable, that the patient will suffer 
greatly at times, and that the final lot of the tabetic is almost 
always a total inability to work or gain a living. In discuss- 
ing the prognosis as to complete recovery, the question arises, 
Is tabes ever curable, or is there even a possibility of cure ? 
This question is, with the proper restrictions, to be answered 
in the affirmative ; it is possible to cure tabes, but only fresh 
cases of luetic origin. Advanced cases, in which degeneration 
in the cord has taken place, are incurable ; we possess no means 
of bringing the lesion to a standstill or causing it to disappear. 
It is evident that the chances for the successful treatment of 
recent cases are increased the younger the patient and the 
better his general constitution. The prognosis is, cceter is pari- 
bus, less favorable in individuals with a neuropathic tendency, 
in whom the disease breaks out in consequence of some ex- 
citing cause, than in fresh specific cases. 

Treatment. — In taking charge of a case of tabes we must 
first see that we ourselves, as well as the patient, clearly under- 
stand how much can be expected from any treatment. If his 
is one of those exceptional cases in which the prognosis is 
relatively favorable, we may tell him so; but in most instances 
it will be our painful duty to make him acquainted with the 
seriousness of the situation, of which he will often be entirely 



680 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

ignorant. We must tell him with gentleness that a complete 
recovery can not be hoped for, and that all that it is possible to 
accomplish is to relieve some of his symptoms and to keep him 
in such a condition that he can as long as possible carry on his 
occupation. There is no disease in which it is more out of 
place to arouse in the patient vain hopes of recovery than in 
tabes. 

The choice of the therapeutic measures themselves depends 
upon the stage of the disease in which we find the patient — 
that is, upon how long he has been sick. In old cases the 
greatest caution ought to be observed, and one should not 
forget that rash therapeutic interference may do more harm 
to the patient than good. The value of a treatment is often 
quite problematical ; its harmfulness is too often quite evident. 
Hygienic and dietetic measures, conscientious nursing and 
cleanliness, injections of morphine in severe attacks of pain, 
occasional cool baths, ever-repeated kindly encouragement, 
these constitute — if we leave out the suspension method, of 
which we shall speak later — the only treatment which old cases 
of tabes need, or, for that matter, can stand. But the recent 
cases also demand a great deal of care and forethought. In 
view of the duration of the treatment, protracted as it will 
probably be, all circumstances have to be taken into account — 
the constitution, the age, the occupation, and, above all, the 
pecuniary situation of the patient. 

As syphilis is at the bottom of so many instances of tabes, 
the question whether we are justified in expecting anything 
from an antisyphilitic treatment should be mooted, but only in 
exceptional cases can we have such a hope — that is, only when 
either signs of syphilis are still present, or when the time that 
has elapsed since their disappearance is relatively short (not 
longer than a few months or at most a year). Such cases are 
very rare ; generally years, perhaps twenty years, will have 
gone by during which the patient has been apparently per- 
fectly well, and then the antisyphilitic treatment is of no avail. 
If, however, we wish to institute it for any reason, possibly be- 
cause the patient himself insists upon it, bold doses ought to 
be given, four, six, even eight grammes (3j-3ij) of potassium 
iodide a day, and from three to six grammes (grs. xlv-3 jss.) of 
mercurial ointment rubbed in daily. In all, two or three hun- 
dred grammes (5vj-Jix) of potassium iodide and the same 
amount of mercurial ointment ought to be used. Recently 



TABES DOR SA LIS. 68 1 

Dinkier, in Erb's clinic, has made careful observations with re- 
gard to the influence and the justification of the treatment by 
mercurial inunctions (Berliner klin. Wochenschr., 1893, 15, 16); 
he comes to the conclusion that in fifty-eight out of seventy- 
one cases — i. e., in about eighty per cent — one or several symp- 
toms were improved by the treatment. 

If we have resolved to try internal medicines, knowing, of 
course, that there is none which acts favorably upon the dis- 
eased nerve elements, we may begin with silver nitrate in doses 
of one centigramme (gr. z / 7 ) in pill form three times a day for 
four or six weeks, after which time it may be combined with 
ergotin (arg. nitr., 0.3 (grs. ivss.) ; extr. secal. corn., 3. (grs. xlv) ; 
pulv. et extr. quass., q. s. ut f. pil. no. 30), of which also one 
pill is to be taken three times a day. Finally, a trial may be 
made with the salicylate of physostigmine, of which one milli- 
gramme (gr. % 6 ) in pill form may be given three times a day 
for a month, as recommended by Meyer in his paper on the 
Influence of Physostigmine upon the Patellar Reflex (Berlin. 
klin. Wochenschr., 1888, 2). With these drugs we ma} 7 be fairly 
confident that we are doing no harm, and often we may per- 
ceive a distinct improvement in the condition of the patient, 
although we are, of course, not able to definitely decide whether 
this is actually to be attributed to the medicine or not. We 
would recommend these remedies more warmly than any other, 
even than strychnine, which has been administered subcutane- 
ously in doses of from three to five milligrammes (gr. V^- 1 /^), 
gradually increased to one centigramme (gr. yl) in twenty-four 
hours, for repeatedly after these injections we have observed 
the occurrence of pains which had not been present for months. 
In the treatment of the individual symptoms we must resort to 
the same measures that we should adopt when these appear in 
the course of other diseases or by themselves ; for instance, for 
the lancinating pains, as in other neuralgias, we shall be obliged 
to give antipyrine and antifebrine, which have recently been 
recommended by Lepine, Suckling, Germain See, G. Fischer, 
and others, but we shall be driven to the conclusion finally, 
that for the relief of these pains there exists only one drug by 
the help of which the patient's painful existence may be ren- 
dered at all bearable — viz., morphine, which here more than 
in any other disease we are justified in using in large amounts. 
The application of a tight bandage to the limb in which the 
lancinating pains are present, as advocated by Leidy (Med. 



682 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

News, August, 1891, 29), I have repeatedly found efficacious; 
the combination of pressure and warmth seems so beneficial to 
many cases. Gastric and laryngeal crises, headache, etc., are 
to be treated symptomatically. 

In addition to the internal medication, it is the electrical 
treatment which deserves special consideration. This, if used 
at a period early enough, may be followed by excellent results, 
and may alone sometimes be capable of effecting a cure or an 
arrest of the morbid process. On the other hand, if we do not 
select our cases properly — for example, if we treat old cases 
like recent ones — we may do more harm than good with it. It 
may give rise to severe pains, and make the patients, who 
until they were treated by electricity were in a fair condition, 
begin to suffer terribly and soon lose confidence in the physi- 
cian. Electricity may also prove successful against the motor 
disturbances, not so much against the ataxia as against the 
weakness in the legs ; also in combating anaesthesias and par- 
aesthesias in the hands and feet it may have some effect, whereas 
it is usually of little avail against the lancinating and rheuma- 
toid pains. How to use the electricity, whether in the form of 
the faradic or the galvanic current, it is impossible to say in a 
few words. Every one forms for himself, in the course of years 
of practice, his own technique, and gives preference to this or 
that method ; the one prefers the galvanic, another the faradic ; 
aofain one will recommend the ascending:, another the descend- 
ing current through the spinal cord ; the one believes in moist, 
the other in dry electrodes, especially the brush. Among all 
the different methods, besides the excellent general faradization 
advised by Beard and Rockwell, the faradic brush applied to 
the back, as recommended by Rumpf, has perhaps met with a 
more favorable reception than any other practice, and justly 
so. We prefer it, so far as electrical treatment goes, to all other 
modes. Details on the subject may be found in my text-book 
on Electro-diagnosis and Electro-therapeutics, in which all the 
points necessary for the practitioner to know are discussed. 

In a large number of cases the cold-water treatment has 
been found to be extremely beneficial. The action of the water 
on the peripheral nerve endings, the influence which cold 
douches, wet packs, moist (" Priessnitz's "•) abdominal bandages, 
cool baths, etc., exert upon the circulation in the vessels of the 
skin, and thus upon the terminal nerve twigs, is often so favor- 
able that marked improvement during and after a stay in a 



TABES DORSALIS. 



68 3 



hydrotherapeutic establishment is not rarely seen. Even in 
cases in which sensory and motor disturbances have attained 
to such a degree that but little can be hoped for, a carefully 
conducted cold-water treatment may be quite beneficial in 
improving the general condition of the patient and raising his 
spirits. 

On the other hand, we would emphatically warn against 
the use of warm or hot as well as steam and sweat baths. As 
a rule they are of no avail, but often evoke the lancinating 
pains. Unfortunately, the physician is not always in a position 
to prevent this, since the patients, who believe implicitly in 
the rheumatic nature of their pains, use them at random with- 
out his orders often for months and years. There are a great 
many tabetics who during the course of their disease have 
taken many hundreds of steam baths without perceiving the 
slightest benefit therefrom. 

From the springs we can, on the whole, expect but little, 
and especially old cases with paraplegia and severe bladder 
troubles should be spared the trial. The disadvantages, the 
overexertion attendant upon the journey, and the lack of home 
comforts, in the case of these patients especially, will far out- 
weigh any good results obtained from the baths ; nor should 
we, as we said above, leave out of sight the necessary cost 
which, even with the most modest pretensions, is not incon- 
siderable. One should never forget that the disease is likely 
to last a very long time, that the patient will soon be unable 
to earn any money, and that for him there can be no greater 
misfortune than to find that, heedlessly or yielding to over- 
persuasion, he has spent all his worldly goods of which now 
he stands in the greatest need. There exist not a few of such 
helpless patients in whose cases just this point was overlooked, 
and it is especially our younger colleagues who seem rather too 
prone to disregard it. If such and other objections do not exist, 
it is most advisable to recommend places where warm brine 
baths can be taken, as in Rehme-Oeynhausen, this place having 
become famous for the treatment of tabes especially, though it 
is my experience that patients get along there no better and no 
worse than at other springs of the same kind — e. g., Nauheim 
— and it only deserves to be warmly recommended Gwing to 
the excellent arrangements which we there find, particularly 
the facilities for moving helpless invalids from place to place. 
Chloride-of-sodium springs containing iodine and bromine — 



684 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

for instance, Konigsdorf-Jastrzemb, Kreuznach, Goczalkowitz, 
Krankenheil — may be tried without fear of doing any harm ; 
while the nonmedicated hot springs of Gastein, Teplitz, Johan- 
nisbad, Warmbrunn, Pfaffers, and the hot sulphur springs of 
Landeck, Aachen, Trentschin, Pistyan, Baden near Vienna, and 
Baden in Switzerland should be prescribed only with great 
caution, and the baths should never be taken too warm, never 
above a temperature of 8o° to 90 F. Among the chalybeate 
springs, first Cudowa, then Pyrmont, Flinsberg, Schwalbach, 
and St. Moritz (Engadine) deserve to be tried. 

The results of massage in the treatment of tabes are not sat- 
isfactory. There is no objection to giving massage in a care- 
ful manner so as to improve the nutrition of the muscles and 
to stimulate metabolism, especially in cases of young, compar- 
atively robust patients ; but we are hardly justified in building 
much upon such a procedure and in expecting to bring about 
a lasting improvement in the sensory or motor disturbances. I 
have known instances in which the general condition of Xhe 
patient was influenced for the worse by massage, and in which 
certain symptoms, especially the lancinating pains, appeared 
to be aggravated after its use. 

Of only historical interest is the operation of nerve stretch- 
ing, which, in the first half of the eighties, was by some claimed 
to be an excellent means in the treatment of tabes, the sciatic 
nerves being usually chosen for this operation. They w r ere laid 
bare by cutting through the gluteal muscles and "stretched" 
according to different methods. The result was in many cases 
at first very striking. Pains, bladder disorders, and anaesthe- 
sias vanished, and the operation was undertaken comparatively 
frequently. Soon, however, it was found that what had been 
regarded as a success was of no long duration, and that the 
old troubles returned, and, finally, after it had been repeatedly 
demonstrated at the autopsy (Striimpell, Rosenstein) that the 
elongation of the nerves not only had not exerted the slightest 
beneficial effect upon the morbid process in the spinal cord, 
but that several times at the place where the nerve had been 
stretched a neuritis had developed and extended to the sub- 
stance of the spinal cord, giving rise to a myelitis, the practice 
was given up, and can be looked upon to-day as having been 
definitely discarded. 

Finally, various other modes of treatment should be men- 
tioned which we may collectively call the mechanical methods. 






TABES DORSALIS. 



685 



Graduated exercises consisting in the execution first of sim- 
ple muscular movements, later of simple co-ordinated, and 
finally of complex co-ordinated movements (Frenkel, Mun- 
chener med. Wochenschr., 1890, 52), it is claimed, bring about 
a decided improvement in the ataxia. Again we have the 
treatment according to the method of Hessing, by which a 
permanent support of the spinal column is attempted ; the 
patient is provided with a corset made of cloth which he has 
to wear for years day and night, and which transfers the 
weight of the body to the 
pelvis and relieves the spinal 
column. Certain clinicians, 
among them Jurgensen (cf. 
lit.) have spoken favorably of 
this procedure, while Miiller, 
of Stuttgart, would prefer 
another form of apparatus, 
since he considers Hessing's 
corset inefficient (Med. Cor- 
respondenzbl., d. Wiirtemb. 
arztl.Landesvereins, 1890, 15). 
Frequent extension of the 
spinal column is attempted 
in the method by suspension 
first advocated by Motschu- 
kowsky (Wratsch, 1883, 17- 
21) and later by Charcot. 
The results obtained with 
this mode of treatment in 
the Salpetriere were favora- 
ble enough to induce many 
clinicians in Germany, Eng- 
land, and America to make 

further trial of it, and at present we possess quite an imposing 
array of articles treating of the " suspension method " and the 
results obtained by it. According to some authors the cere- 
bral, according to others the spinal symptoms are improved 
by it. The procedure is said to be without danger, but in one 
instance the immediate consequences were fatal ; it should be 
said, however, that in this case the suspension was undertaken 
without the physician's supervision (Gorecki, Lyon med., 1889, 
20). Althaus (cf. lit.) has attempted to give an explanation of 




Fig. 181.— Suspension Apparatus used in 
the Treatment of Tabes. 



686 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

the mode of action of this treatment. According to his opinion, 
the meningitic adhesions over the posterior columns are loos- 
ened, so that the nerve fibres, especially the superficial ones, 
gain in power of conduction, the sclerosed, thickened neuroglia 
becomes looser, and the pressure upon the nerve tubes is thus 
diminished. He also thinks that suspension should only be 
used in older cases, because in recent ones it might lead to 
inflammatory conditions. The possibility that this loosening 
does take place, as Althaus claims, can not be disproved, but 
this much is certain, that for those instances in which improve- 
ment is said to have shown itself after only one, two, three, 
or ten suspensions, this theory affords no explanation. 

The results which I have seen from the method by sus- 
pension both in my clinic and in private practice are by no 
means encouraging. Outside of an improvement in vision, 
which I have been able to note and which Bechterew (Neurol. 
CentralbL, 1893, 18) also observed, I have not in a single in- 
stance been able to persuade myself that any marked or lasting 
improvement took place. The account wmich intelligent and 
unprejudiced patients gave of themselves after the thirtieth, 
fiftieth, and eightieth suspension corresponded almost exactly 
to that which they had given prior to the institution of the 
treatment. In opposition to Althaus, then, it is my conviction 
that anatomical changes are not produced by suspension, but 
that the transient improvement has to be referred to the influ- 
ence of suggestion. The patients hear of a new treatment for 
their incurable disease, they subject themselves to it with much 
pleasure and confidence, and by autosuggestion produce an 
improvement in some functional impediment (for example, in 
the ataxia), which may be quite marked, but which is never 
lasting. Four times during the act of suspension I myself. met 
with rather unpleasant accidents ; in two cases the patients 
lost consciousness and had to be rapidly taken down, and were 
only then with some difficulty recalled to life ; in two other 
instances severe laryngeal crises appeared, so that the pro- 
cedure had at once to be stopped. Such accidents, of course, 
make a very bad impression upon the patient, and bring the 
results, which are in any case doubtful, still more into ques- 
tion. Careful examination is necessary before the suspension 
is used, and if there exists a disease of the heart or of the ves- 
sels it should under no consideration be undertaken. We need, 
of course, hardly add that while the patient is suspended he 



TABES DOR SALTS. 



687 



should be carefully watched. Benuzzi has recently attempted 
to replace the suspension by simple stretching, and claims that 
with his method the spinal cord is extended much more de- 
cidedly. The legs are held at the ankle joints with a towel 
and are pulled over the head until the knees touch the fore- 
head. Benedikt has seen good results from this method in a 
number of cases (Wiener med. Presse, 1892, 1). I myself soon 
abandoned the procedure, owing to the fact that it is very dis- 
agreeable to most patients, I must admit, however, that it is 
deserving of further trial in suitable cases. 

LITERATURE. 

8. Treatment. 

Jiirgensen. Ueber die mechanische Behandlung der Tabes nach dem System 

Hessing. Deutsche med. Wochenschr., 1889, 40. 
Leyden. Berliner klin. Wochenschr., 1892, 17, 18. 
Laborde. Du traitement electrique du tabes. These de Bordeaux, 1893. 

Treatment by "Suspension." 

Charcot. De la suspension dans le traitement de l'ataxie locomotrice progres- 
sive et de quelques autres maladies du systeme nerveux. Progres med., 
1889, 3. 

Weir Mitchell. Med. News, April 13, 1889. 

Chernel. Wiener med. Blatter, 1889, xii, 5. 

Dana. New York Medical Record, April 15, 1889, xxxv. 

Gilles de la Tourette. Progres med., 1889, xvii, 8. (Technique of Suspension.) 

Morton. New York Med. Record, April 15, 1889, xxxv. 

Althaus. Lancet, April 13, 1889, p. 160. 

Wattewille. On the Treatment by Suspension of Locomotor Ataxy. London, 
1888. 

v. Openchowski. German translation of Motshukowski's investigations. Berliner 
klin. Wochenschr., 1889, 25. 

Benedikt. Wiener med. Wochenschr., 1889, 45, 46. 

Haushalter et Adam. Progres med., 1889, 44. 

Gilles de la Tourette. Arch, de Neurol, Juillet, 1889, xviii, No. 52. 

Pincherli. Rivista veneta di scienze med., 1889, Ottobre. 

Rosenbaum. Deutsche Med.-Ztg., 1890, 39, p. 444. 

Lohmann. Ibid., 1890, 69. 

Cagney. Lancet, January 18, 1890. 



CHAPTER III. 

DEMENTIA PARALYTICA PROGRESSIVA — GENERAL PARALYSIS OF THE 
INSANE GENERAL PARESIS SOFTENING OF THE BRAIN. 

While in tabes we have learned to recognize a disease of 
the general nervous system, in which the spinal cord chiefly is 
the affected portion, we find that in dementia paralytica, on the 
other hand, the brain is pre-eminently the part attacked, whereas 
the spinal cord and the peripheral nerves do not suffer so regu- 
larly nor to so marked a degree. In its distribution the lesion 
of the cord is either diffuse or, as in tabes, confined to the pos- 
terior columns. With regard to the affections of the spinal 
nerves a more careful study is still necessary, and more espe- 
cially this question needs to be answered whether here, as 
seems indeed very probable, primary degenerative processes, 
analogous to those of tabes, can also be demonstrated. 

iEtiology. — The manifold points of resemblance between 
general paralysis and tabes, to some of which we have drawn 
attention above, are found first in the aetiology of the two dis- 
eases. In the former as well as in the latter hereditary tenden- 
cies are of much significance. A person belonging to a family 
in which nervous diseases of any kind have been prevalent is 
more prone to develop general paralysis than a member of a 
healthy family. However, this factor is in the majority of in- 
stances not sufficient in itself to bring about the disease, certain 
exciting causes being also necessary, and experience has taught 
us that it is chiefly overstrain of the nervous system, and more 
especially of the brain, which favors its development. Such 
excessive demands which are made upon the brain are numer- 
ous. Chief among them is mental overstrain, caused by too 
close attention to work and worrying over business — too much 
scheming, calculating, etc. — so that we are not surprised that 
bookkeepers, accountants, bankers, stock brokers, authors, 
actors, etc., form the relatively largest contingent of cases. 
Besides this, deep emotions, repeated or long-lasting sorrows 
or care, the struggle for existence, disappointed hopes, baffled 

688 



DEMENTIA PARALYTICA. 



689 



ambitions, and fright, may attain an serological importance. 
On hearing the history of a general paralytic, one at least of 
these factors will hardly ever be absent. Bodily overstrain, 
forced marches, excesses in Venere and the like, can be made 
responsible to a less extent. Excesses in BaccJw, the habitual 
abuse of alcohol, only rarely lead to general paralysis, but 
sometimes a condition is produced by such excesses, the " pseudo- 
paralysis a potu," which resembles general paralysis, but which 
is quite distinct from it, and belongs to chronic alcoholism. 
There is no question but that the occupation may furnish 
causes for the disease ; thus, those which entail at once bodily 
and mental work, or those in which the workers must for 
a long time remain in very hot rooms, and again working in 
poisons, especially in lead (Snell, Vogel, and others), are par- 
ticularly dangerous. Of great interest, finally, is the fact that, 
like tabes, general paralysis may be caused by traumatism 
either to the head or the back, so that we have a traumatic 
progressive paralysis which is quite analogous to the traumatic 
tabes. In this latter category we must also place the insolation, 
(sunstroke, heat stroke) which has been known to lead to gen- 
eral paralysis (Bonnet and Paris, Ann. med. -psych., Novembre, 
1834, 6, s. 12). 

Besides the congenital, however, there exists also an ac- 
quired predisposition, which differs from the former, inasmuch 
as no other exciting causes are needed for the production of 
the disease, since, just as is the case in tabes, it alone is suffi- 
cient to bring about general paralysis. We refer, of course, to 
syphilis. The same highly important role which it plays in 
tabes it plays here too. An individual who has had syphilis 
has much greater cause to fear general paralysis than one who 
has never been infected. According to the statistics of Rieger 
(cf. lit.), the one is sixteen or seventeen times as liable to the 
disease as the other. These figures correspond very closely 
with those founded on my own experience. Out of two hun- 
dred and fifty-seven paralytics a hundred and seventy-one had 
been syphilitic, and out of two hundred and sixty patients with 
other diseases only fourteen. Heredity and all the exciting 
causes taken together do not give rise to as many cases of pa- 
ralysis as does syphilis alone ; but here again, as in tabes, we 
must leave the question open as to how syphilis acts, whether, 
as I myself am inclined to think, the syphilitic arterial disease 
is responsible, or whether we are dealing with a toxic action 
44 



690 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

so that general paralysis has to be regarded as a post-syphilitic 
affection. Whatever our decision on this point, the fact that 
general paralysis may be the result of syphilis is universally 
acknowledged, and the numerous writings which we possess 
on the subject are all without exception in favor of this view. 

The influence which has been ascribed to age and sex can 
usually be explained by that of syphilis. Males are more fre- 
quently attacked than females, the ratio being seven men to 
two women. Those in the prime of life furnish the largest 
contingent. 

Symptoms. — The symptoms of the disease are partly psy- 
chical, partly somatic, and this will not surprise us when we 
learn that the seat of the affection is preferably in the brain, 
and more particularly in the psycho-motor region of the cere- 
bral cortex. The psychical manifestations differ very greatly, 
and it is more especially in the prodromal stage that these 
variations are most noticeable. This is a feature equally well 
marked here as in tabes, and the main difference between the 
two consists in the fact that in general paralysis the clinical 
picture of the prodromal stage is dominated by the psychical 
manifestations. The patient becomes unable to concentrate 
his mind for any length of time. He gets easily fatigued when 
he has exerted himself mentally, he becomes forgetful, and is 
no longer able to comprehend and deal with matters which he 
previously understood perfectly. He is found to be indifferent 
in the performance of his duties and careless in keeping his ap- 
pointments ; he becomes unreliable and absent-minded. When 
Avriting, he makes mistakes in spelling, and presents a slowness 
in thinking and a general dullness of intellect which are quite 
foreign to him. At the same time his disposition presents 
alterations. Previously tolerant and kind, he becomes now ill- 
humored, moody, and irritable ; on the slightest provocation 
he loses his temper and may even be inclined to violence. His 
character is not the same as it was ; his will power becomes 
weak ; he loses his energy and his moral individuality ; he 
allows himself to be influenced and overpersuaded by anybody, 
and even thus early does things for which he can give no clear 
motive ; he gradually loses all consideration for others in his 
social intercourse ; he neglects his appearance, his dress looks 
untidy, he becomes indecent, commits nuisances on the open 
streets, tells obscene stories before his children, and so forth. 



DEM EN TIA PARALY TIC A . 



691 



In exceptional cases the patient himself is to a certain extent 
conscious of these changes which are going on in him. They 
surprise him, and he speaks about them to his most intimate 
friends and expresses a fear that some serious disease is coming 
on ; but in the great majority of cases, he does not in the least 
appreciate his condition, which worries and troubles his family 
so much. Months, even years, may thus pass and no new mani- 
festations make their appearance. It is only the occurrence of 
certain somatic symptoms which gives to the clinical picture 
a different aspect. Among these latter, besides a very trouble- 
some ophthalmic migraine, which is frequently observed, there 
are especially two on account of which the physician is consulted, 
namely, insomnia and the alteration in speech. The former 
is all the more striking because the patients often by day and 
at their work are overpowered by sleep, while at night they 
lie awake for hours without being able to rest. The latter 
manifests itself by a difficulty in pronouncing certain words. 
The patient stutters, misplaces letters and syllables, leaves syl- 
lables out; in a word, presents the group of symptoms known 
as " syllable stumbling " (Sylbenstolpern). At the same time the 
voice loses its usual timbre ; it becomes harsh and its former 
modulation is gone. 

For the examining physician, the associated movements in 
the facial muscles, the fibrillary tremor and twitching of the 
lips, and the tremulousness of the protruded tongue are suffi- 
cient to lead him to the diagnosis, and the inequality of the 
pupils which may appear at this stage is an important sign. 
Ballet has shown that other ocular symptoms may be utilized 
(Progres med., 1893, 23 ; cf. also Oebecke, Allgem. Zeitschr., f. 
Psych., 1893, Heft 1, 2, p. 169). The motor disturbances (Le- 
moine et Lecordonnier, Gaz. med. de Paris, 1889, November 2) 
further manifest themselves in a change in the handwriting and 
in the gait. The writing shows uncertainty and irregularity ; 
the letters, which are usually larger and written more awk- 
wardly than before, become tremulous ; the paper is covered 
with blots ; the words are incorrectly written, inasmuch as 
letters or entire syllables are omitted or misplaced. The gait 
becomes awkward and clumsy and the patient " shuffles along " ; 
he is one-sided, and small obstacles in his path are apt to cause 
him to fall. 

This initial stage, which in its duration varies from a few 
months to one, even two or three years, is followed by a stage 



6g 2 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

which is generally characterized by a rapid increase in the 
psychical excitement (" maniacal exaltation "). The patient — 
previously quiet, sullen, apparently occupied with his own 
thoughts — now becomes noisy, talkative, all the time restless 
and in a state of excitement ; without noticing his surround- 
ings and his friends, he lives with a sense of perfect comfort ; 
he is young, handsome, extremely strong, and immensely rich ; 
he has studied all sciences ; he occupies himself with absolutely 
preposterous but to him feasible schemes ; he is going to dry 
up the Atlantic Ocean, he is the Emperor of China, he is Na- 
poleon, Christ, he is the chief among the gods, etc. In the 
dreamlike play of his imagination all these fantasies arise, but 
the patient is not able to give them any logical connection. 
Without critical faculty he stands out a pitiable victim of the 
most bizarre delusions of grandeur. At the same time his 
memory rapidly fails him, especially for recent events ; what 
he did to-day, yesterday, the last visit of the physician, etc., he 
does not remember, whereas the reminiscences of long-past 
years can still be called up. He does not know the day of the 
week or even the name of the month and the season in which 
he is at present. People with whom he used to deal in busi- 
ness he no longer recognizes ; he confounds them with other 
persons, etc. The lack of judgment of the patient has, of 
course, a decided influence upon his actions ; he buys things 
recklessly, squanders his money in a most foolish manner, he 
makes debts, commits easily discoverable frauds, which he de- 
nies with the utmost calmness when he is found out. Assaults 
of which he may be guilty, misdemeanors against the public 
order, offenses against the public morals, etc., not infrequently 
lead to trouble with the authorities and to the arrest of the 
patient. 

In by far the smaller number of cases the above-described 
initial stage is followed, instead of by the maniacal exaltation, 
by a stage of depression. The patient believes himself perse- 
cuted by everybody, and his life menaced ; he hears voices, and 
he is always troubled with a presentiment that something ter- 
rible is going to happen. He cries, laments, begs for help, and 
so forth. In other instances hypochondriacal delusions gain 
the upper hand. The patient imagines that he is made of 
glass, that he can not eat, that he is unable to urinate, that he 
has no head, and the like. The lack of all power of criticism 
in these delusions, and the inability to systematically elaborate 



DEMENTIA PARALYTICA. 



693 



them, and the usually rapid course of this stage, distinguish the 
general paralytic from the paranoiac. 

Quite gradually in the course of time the general aspect 
changes, the excitement abates and disappears, and the intel- 
lectual impotency increases. The patient spends his days with- 
out a thought or care, writing and reading become to him lost 
arts, he forgets his own name, and his social position, he be- 
comes oblivious of his family, and in general takes no interest 
whatever in the outside world. This is the stage of dementia. 
He becomes uncleanly in his habits, his eating and drinking 
must be watched, and step by step the psychical life approaches 
more and more its extinction ; the patient no longer lives, he 
vegetates. 

It is of great practical importance and interest to study 
the somatic disturbances which occur in the course of the dis- 
ease associated with the psychical ones, and which are caused 
by the simultaneous affection of the spinal cord (and periph- 
eral nerves). The diminution of sensibility, that of the skin as 
well as of the nerves of special sense, particularly of the opticus, 
the absolute inactivity of the pupils, the decrease in the per- 
ception of pain, the changes in the electrical excitability of the 
muscles, which at first is increased, later diminished, the (not 
regular) loss of the tendon reflexes, the appearance of trophic 
disturbances (ichthyosis, Fere), the tendency to bedsores, the 
perforating ulcer of the foot (ma/ per for ant dn pied, cf. page 
656), all point to a participation of the spinal cord in the mor- 
bid process. Sometimes, quite early, peculiar attacks occur, 
which, associated with loss of consciousness, are either accom- 
panied by transient hemiplegias or convulsive movements, and 
which therefore either deserve the name of apoplectiform or 
epileptiform seizures. They are designated as " paralytic at- 
tacks." Under certain circumstances they appear very fre- 
quently, from ten to fifty times in one day, and they may then 
keep the patient in an almost constant condition of uncon- 
sciousness. The elevation of temperature which accompanies 
these attacks is not considerable, the occurrence of albumin 
in the urine not constant. Among the affections of the cranial 
nerves which have been but little studied in their connec- 
tion with general paralysis may be mentioned more particu- 
larly the optic atrophy, which is seen in ten per cent of all 
cases. The nerves of the ocular muscles also frequently be- 
come involved, the implication of the trigeminus and of the 



694 -DISEASES OF THE GENERAL NERVOUS SYSTEM. 

facial being less common. Of the nuclear affections of the 
vagus coming on in the course of this paralysis nothing defi- 
nite is known. 

The duration of the disease varies much. In the " galloping 
form," in which, owing to the sleeplessness and inability to take 
sufficient food, the strength rapidly fails, it may require only a 
few months to bring about a fatal issue. At other times the 
disease may last two, three, five, or even more years, out of 
which no small proportion is liable to be spent in an asylum, 
as it is out of the question to keep the patient at home, in 
spite of all the care and devotion possible on the part of the 
family. 

Pathological Anatomy. — The questions as to the anatom- 
ical nature of the disease have unfortunately not been as yet 
answered satisfactorily, and there is still a great deal of diver- 
sity of opinion among the authorities on this point, although 
the macroscopical appearances are usually very characteristic, 
the atrophy of the brain, especially in the anterior regions, 
being very striking. Although no one can doubt that the con- 
volutions are diminished in size, that the frontal and the parie- 
tal lobes weigh less than in a normal brain, yet the precise 
mode in which this atrophy comes about, what are the micro- 
scopical changes in the nerve elements of the cortex, and what 
is the primary process in all this, are not as yet decided, but re- 
main the subject of much controversy. According to Tuczek, 
there is a marked primary atrophy of the fine medullated nerve 
fibres, particularly in the outer layers of the cortex, in the 
tangential " association " fibres, which run parallel to the sur- 
face. The gyrus rectus is said to be relatively the earliest 
attacked, later, the remaining frontal brain and the island of 
Reil, then the temporal, but the occipital lobes never. This 
view, according to w T hich the atrophy is the primary process, 
is in all probability correct, although it is still combated by 
some authorities (Mendel), who look upon the death of the 
nerve fibres as the secondary, upon the increase of the in- 
terstitial tissue, the thickening of the vessel walls, and the ap- 
pearance of spider cells, as the primary process (" encephalitis 
interstitialis "). 

Analogous changes in the ganglionic cells have frequently 
been noted (Binswanger, Mendel, Gudden) ; a peculiar aggre- 
gation of nuclei associated with disease of the vessels, degener- 
ation of the capillaries (Kronthal, Neurol. Centralbl., 1890, 22), 



DEM EN TIA PARALY TIC A . 



695 



changes in the bodies of the cells in the large pyramids of the 
paracentral lobule, changes in the nucleoli and nuclei, and 
sclerosis and atrophy of the cells are not uncommonly found 
in this connection. 

But, besides the cortex, the deeper regions also are the 
seat of alterations, and the manifold changes which the white 
matter of the hemispheres may undergo, have been studied 
among others by Friedmann. He describes four different 
forms of atrophy of the fibres of the white matter, the number 
of the fibres diminishing in a manner analogous to that which 
has been shown by Tuczek to be true for the cortex. The 
central ganglia of the brain do not remain exempt. Lissauer 
describes a degeneration extending from behind forward, by 
which the pulvinar is often only partially implicated, the in- 
ternal geniculate body sometimes, the external geniculate 
body never ; he is of opinion that this degeneration is pres- 
ent in cases in which well-marked sensory focal symptoms 
accompany the paralytic attacks, but admits that these changes 
in the thalamus are by no means constant. Westphal has 
shown that the pyramidal tracts or the posterior columns 
of the spinal cord are also often affected, a fact which prob- 
ably accounts for a not inconsiderable part of the motor 
disturbances. 

The condition of the pia varies. Frequently it is adherent 
over large areas of the underlying cortex, so that it can not 
be stripped off without loss of substance (" decortication "). In 
rare instances, although it is nowhere adherent, in places it 
is thickened, of greater consistence than normal, and contains 
variable amounts of fluid in its meshes. Whether the latter 
condition is only a later stage of the former — that is, whether 
adhesions only exist at first, but later disappear — is not defi- 
nitely known. 

A case reported by Rey (cf. lit.) shows that exceptionally 
all the sy mptoms of progressive paralysis of the insane may be 
observed during life, and yet at the autopsy no change be found. 
The same thing, as we have mentioned, has been known to 
occur in connection with multiple sclerosis. 

Diagnosis. — The diagnosis may present some difficulty, inas- 
much as in certain forms of chronic alcoholism the egoism may 
be exaggerated as in general paralysis, and inasmuch as cere- 
bral syphilis, brain tumor, senile dementia, finally, chronic 
meningitis, especially the diffuse syphilitic basal form (Oppen- 



696 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

heim), and multiple sclerosis, may more or less resemble gen- 
eral paralysis in their course and their symptoms. In alcohol- 
ism the hallucinations are wont to be a prominent feature, the 
speech disturbances are less marked, and the ideas are worked 
out in a more connected manner. The tremor and the history 
in cases of chronic alcoholism will also assist us in our diagnosis. 
In cerebral syphilis also the history as well as the age of the 
patient (who is, as a rule, younger than the paralytic) must be 
taken into consideration. Brain tumors present a similarly 
progressive course, but the stage of exaltation is absent and 
the characteristic delusions of grandeur do not occur ; in 
place of them we have stupor and somnolence. Senile de- 
mentia, of course, occurs in people of advanced age, and is 
characterized by a tendency of the process to remain stationary 
for some time. 

In meningitis we have febrile symptoms ; the choked disks, 
which are found comparatively frequently here, and the de- 
lirium which occurs early will guard us against errors. In 
multiple sclerosis, finally, we have the scanning speech and 
the intention tremor, and when the disease is well developed, 
it can not be mistaken for general paralysis. In certain forms, 
however, the differentiation may be impossible. The most im- 
portant points to be remembered in the diagnosis of general 
paralysis are, then, the following : The pronounced psychical 
weakness, which even in the initial stages is the most promi- 
nent feature of the disease ; the constantly progressive course ; 
the motor as well as the sensory changes, the former of which 
give rise to more or less marked alterations in the speech, the 
handwriting, and the walk, the latter to changes in the im- 
pressionability to external stimuli and to marked interference 
with the functions of the nerves of special sense — the cutaneous 
sensibility, the sense of taste, hearing, and smell. With-this in 
mind we shall make a correct diagnosis at least in a good many 
cases ; to avoid errors completely will be impossible even to 
the most experienced. 

Prognosis. — We need hardly say much about the prognosis. 
From the above description we can well infer how unfavorable 
it must be. Almost all cases prove fatal in a few years, and 
the outlook for complete recovery is worse here than in tabes. 
To be sure, it has been claimed that such may occur in pro- 
gressive paralysis (Wendt, Voisin), but, in the instances in 
which it was observed, the possibility that the case was not 



DEM EX TIA PARALY TIC A . 



697 



one of dementia paralytica, but rather one of the so-called 
pseudo-paralyses, such as are known to occur after the abuse 
of alcohol, can not be excluded with certainty. 

Treatment. — In the treatment of the disease we must chiefly 
endeavor to keep away all excitement from the patient, and, 
since this is best and most easily accomplished in an asylum, it 
is the first duty of the physician, after he has once made the 
diagnosis definitely, to urge the family to transfer the patient to 
some such institution. Only then is it possible to guard the 
patient as well as the family against all the accidents and fatali- 
ties to which he is otherwise necessarily exposed. This step 
must be taken as early as possible, not with the idea that the 
patient will be cured, but with the conviction that only in an 
institution is he safe, and that there alone it will be possible to 
secure for him the proper care and nursing so necessary for 
one in his condition. Where there is a history of syphilis, the 
treatment with inunctions must of necessity be given a trial, 
however slight may be the prospect of success. Once decided 
upon, let the antisyphilitic treatment be pursued with vigor ; 
at least three to four hundred grammes (Jix-gxij) of mer- 
curial ointment should be used altogether, to which must be 
added from two to three grammes (grs. xxx-xlv) of the iodide 
of potassium daily for a good while. The chloride of gold and 
sodium, a remedy which years ago was highly esteemed for its 
antisyphilitic action, has again been brought back from oblivion 
and used in cases of general paralysis (Boubila, Hadjes, and 
Cossa, Annal. med. -psych., 1892, 1, 2) ; the results are not better 
than those obtained with any other drug. To meet the out- 
breaks of exaltation and the insomnia the usual hypnotics, 
which are, however, of little avail, should be tried. Sulphonal 
in doses of two or three grammes (grs. xxx-xlv), methylal in 
doses of from five to eight grammes (grs. lxxv-3ij), by the 
mouth (Mairet and Combemale), morphine, from one and a 
half to three centigrammes (grs. %— %) hypodermically, chloral, 
paraldehyde, possibly also hyoscyamine, should be tried in 
turn. The cold-water treatment and baths, also galvanism to 
the brain, are decidedly contraindicated. All such procedures 
are likely only to increase the excitability of the patient, to 
give him all kinds of unpleasant sensations, and to make his 
troubles worse, without being in any way of benefit to him or 
relieving his condition. 



698 DISEASES OF THE GENERAL NERVOUS SYSTEM. 

LITERATURE. 

a. Symptoms. 

Striimpell. Neurolog. Centralblatt, 1888, 5. (General Paralysis combined with 

Tabes in a Child after Syphilis of the Father.) 
Jelly. Boston Med. and Surg. Journ., July 2, 1888, cxix. (Duration of Ten 

Years.) 
Rottenbiller. Centralbl. f. Nervenheilkunde, 1889, xii, 1. (Observations on the 

Temperature.) 
Buchholz. Das Verhalten der Pupillen bei Dementia paralytica. Inaug.-Dissert., 

Breslau, 1889. 
Wendt. Allgem. Zeitschrift f. Psych., 1889, xlvi, 1. (Recovery.) 
Voisin. Bull, de therapeut, Mai 15, 1889. (Curability of General Paralysis.) 
Gerlach. Arch. f. Psych, u. Nervenkh., 1889, xx, 3. (Changes in the Galvanic 

Excitability.) 
Ascher. Allgem. Zeitschr. f. Psych., 1889, xlvi, 1. (Course and ^Etiology of 

General Paralysis.) 
Godet. Revue med. de la Suisse rom., April, 1889, ix, 4. (Pseudo-paralysis 

caused by Alcoholic Excesses.) 
Meyer und Weber. Peptonurie bei allgemeiner Paralyse. Bericht iiber die Ir- 

renanstalt, Basel, 1889. 
Blocq. Arch, de Neurol., November, 1889. 

Ascher. Aphasie bei allgemeiner Paralyse. Allg. Zeitschr. f. Psych., 1892, 1, 2. 
Redlich. Zur Charakteristik der reflectorischen Pupillenstarre bei progressiver 

Paralyse. Neurol. Centralbl., 1892, 10. 
Nacke. Allg. Zeitschr. f. Psych., 1892, 1, 2. (Katatonic Symptoms.) 
Konig. Ibid., 1892, 1, 2. (Transient Speech Disturbances.) 
Neisser. Ibid., 1892, 3. (Combination of General Paralysis and Progressive 

Muscular Atrophy.) 
Raymond, F. Semaine med., 1892, 25. (Relation of General Paralysis to 

Tabes.) 
Rendu. Ibid., 1892, 31. 
Pierret. Ibid., 1892, 41. 

Nageotte. Tabes et paralysie generate. Paris, 1893, Steinheil. 
Marie, P. Extraits des Bulletins de la Soc. med. des hop. de Paris. Seance du 

12 Janv. 1894. 

b. Pathological Anatomy. 

Tuczek. Beitrag zur pathologischen Anatomie und Pathologie der Paralyse. 

Berlin, Hirschwald, 1884. 
Zacher. Arch. f. Psych, u. Nervenkh., 1884, xv, 2. 
Savage. Journ. of Mental Sc, January, 1884, xxix. (Pachymeningitis and 

Paralysis.) 
Camuset. Annal. medico-psychol., Novembre, 1884, 6me ser., xii. (Changes 

of the Dura Mater in General Paralysis.) 
Mendel. Allgem. Zeitschr. f. Psych., 1885, xii, 4, 5. (The Ganglionic Cells of 

the Brain Cortex in General Paralysis.) 
Baillarger. Ann. med.-psychol, 1886, xliv, 1. (Diminution in Weight of the 

Cerebrum.) 



DEMENTIA PARALYTICA. 



699 



Meynert. Vom Mechanismus der progressiven Paralyse. Wiener med. Blatter, 
1887, x, 17, 18. 

Zacher. Das Verhalten der markhaltigen Nervenfasern u. s. w. Arch. f. Psych, 
u. Nervenkh., 1887, xviii, 1. 

Zacher. Arch. f. Psych, u. Nervenkh., 1888, xix, 3. (General Paralysis asso- 
ciated with Focal Lesions in the Internal Capsule.) 

Rey. Ann. med. -psych., Mars, 1889, 7me ser., ix, 2. (During Life Symptoms 
of General Paralysis ; at the Autopsy no Changes found.) 

Binswanger. Allg. Zeitschr. f. Psych., 1886, xlii, 4. (Pathological Histology.) 

Cotella, Rosolino. Le alterazioni dei nervi periferici nella paralisi generale' pro- 
gressiva in rapporto con i loro nuclei centrali di origine. Annali di Nevro- 
logia, Nuova Seria, 1891, ix. 

Hoche. Beitrage zur Kenntniss des anatomischen Verhaltens der menschlichen 
Ruckenmarkswurzeln bei der Dementia paralytica. Heidelberg, Horning, 
1892. 

Joffroy. Contribution a. l'Anatomie pathologique de la Paralysie gener. Arch, 
de med. exp. et d'Anat. pathol., 1892, p. 841. 

c. JEtiology and Occurrence. 

Thomsen. Die praktische Bedeutung der Syphilis-Paralysefrage. Allg. Zeitschr. 
f. Psych., 1890, xlvi, 5. 

Bouchaud. Annal. med. psychol., 1891, 7 ser., xiii, 3. 

Meilhon. Ibid., 1891. 

Bonnet. Rapports de la Syphilis et de la Paralysie generale. These de Paris, 
1891. 

Charcot et Blocq. Paralysie generale chez l'adolescent. Semaine med., 
1892, 6. 

Regis. Syphilis et paralysie generale. Arch, cliniques de Bord., 1892, i, 7, 8. 

Oebeke. Zur Aetiologie der Paralyse. Allg. Zeitschr. f. Psych., 1892, 1, 2. 

Pierson. Bericht uber die Privatheilanstalt u. s. w. zu Pirna, Dresden, 1893. 

Morel-Lavallee. Revue de med., 1893, 2. (Syphilis and General Paralysis.) 

Niconleau. Annal. med. -psych., 1893, 7. 8. (Causes of General Paralysis.) 

Wiglesworth. British Med. Journ., March, 1893, 25. (General Paralysis dur- 
ing Puberty.) 

Fournier. Syphilis et paralysie generale. Revue neurol., 1893, 10. 

Westphal, A. Charite-Annalen, 1893, p. 719. (General Paralysis in Women.) 

Chanson. Essai sur les rapports de la paralysie generale et Syphilis. These 
de Paris, 1893. 



CHAPTER IV. 

SYPHILIS OF -THE GENERAL NERVOUS SYSTEM. 

In different places in our book, in the chapters on diseases 
of the brain as well as in those treating of affections of the 
spinal cord, we have had occasion to point out the role which 
syphilis plays as an ^etiological factor in various diseases. We 
have also shown that tabes and the progressive paralysis of the 
insane are to be regarded as the main representatives of affec- 
tions of the general nervous system depending upon syphilitic 
infection. It only remains, therefore, in the present chapter to 
add some general remarks to what has already been stated. 

No part of the nervous system, whether of the brain or of 
the spinal cord, is exempt from the chance of becoming impli- 
cated in the syphilitic infection, and remembering how the 
blood-vessels are affected by syphilis this is easily understood. 
Clinically, it is especially interesting if we are able to recognize 
diseases of the cerebral cortex and symptoms — e. g., monople- 
gias — resulting therefrom, as syphilitic, but the corona radiata 
and the basal ganglia, the pons, the medulla oblongata, and the 
cerebellum, all may become the seat of syphilis, and syphilitic 
affections of the base of the brain are relatively common. In 
many cases it is difficult to make a certain diagnosis, especially 
if the patient denies the primary sore and no trace of it can 
be found, for the clinical symptoms, of course, are the same, 
whether the brain lesion depends upon syphilis or not. 

Symptoms. — Among the manifold symptoms which occur 
in brain syphilis we may mention polyuria and polydipsia, 
which have been subjected to careful study (cf. lit.). If focal 
symptoms are present it is easier to make a diagnosis than in 
their absence. In the latter case it may sometimes be impos- 
sible to decide upon the diagnosis of brain syphilis ; we may 
be dealing with a case of cerebral neurasthenia. 

With reference to the spinal cord the matter is somewhat 
more simple, because syphilitic disease here, which does not 
implicate also the brain, as in tabes and general paralysis, is 
700 



SYPHILIS OF THE GENERAL NERVOUS SYSTEM. 70 1 

rather rare. It is not a common thing to find disease of one 
system of fibres or disease of several systems combined de- 
pending upon syphilis, and the cases in which lateral sclerosis, 
for example, was attributed to this have been published as 
rarities. It is of pathological interest to note that the root 
bundles usually present a marked and extensive participation 
in the process. In a case reported by Siemerling (cf. lit.) there 
were gummatous growths of the pia, which, although they had 
extended into the substance of the cord, had not attacked any 
" system " in its whole extent, so that, as is often the case, the 
spinal symptoms here also were not at all prominent. 

We can not assume that the spinal nerves, either motor or 
sensory, ever become diseased alone, but we must rather look 
upon their implication as a partial manifestation of a general 
affection. If in exceptional cases we find a neuritis of the 
sciatic or of the musculo-spiral nerve, etc., which we have to 
regard as of syphilitic origin, perhaps because it rapidly passes 
off under antisyphilitic treatment, the manifestations of cerebral 
and spinal syphilis have either existed previously and have not 
been recognized or their presence later has to be looked for. 

Diagnosis. — The diagnosis is based first upon the history of 
the patient and the presence of signs of the primary sore. If 
these are established, it is relatively easy ; if not, we must look 
for other signs to help us. Secondly, the other organs — for 
instance, the skin, the visible mucous membranes — all must be 
examined for the possible existence of syphilitic lesions. Re- 
peated and careful search may sometimes clear up much that 
is obscure, although the patient's account may be imperfect. 
Thirdly, it must be remembered that the symptoms of cerebral 
syphilis are extremely changeable, and are rarely ever of long 
duration. To-day, matters may look as if the patient's life 
were in danger, while to-morrow he is apparently perfectly 
safe again. The rapidity with which the changes in the con- 
dition follow each other, just as in hysteria, the extraordinary 
circumstance that apoplectiform attacks occur in younger and 
epileptiform attacks in older persons, in doubtful cases are in 
favor of the diagnosis of syphilis of the nervous system. In 
every instance we shall do well to pay careful attention to the 
condition of the eye-muscles (Uhthoff, Arch. f. Ophthalm., 1893, 
1) and the pupillary reaction ; the latter may temporarily dis- 
appear and reappear; the same peculiarity may be found in 
the condition of the patellar reflexes, an anatomical explanation 



702 



DISEASES OF THE GENERAL NERVOUS SYSTEM. 



for which has thus far not been arrived at. Finally, the thera- 
peutic test is of some value, inasmuch as the successful anti- 
syphilitic treatment makes the existence of syphilis almost 
certain, although a failure does not warrant the contrary con- 
clusion. 

Prognosis. — The prognosis must, above all, be influenced by 
the time which has elapsed since the primary infection and be- 
fore the first appearance of the nervous symptoms. The longer 
this period of incubation the worse is the prognosis. Accord- 
ing to my own experience, from five to nine years is the most 
common time. Occasionally the infection of the nervous sys- 
tem manifests itself earlier, and in quite exceptional instances 
two years, or even one year, after the primary lesion ; but often 
the interval which elapses is longer than the time above given. 
Cases in which the spinal or cerebral symptoms did not appear 
till after twenty or twenty-five years I have never seen get 
well. The second question of importance is, how long the 
nervous symptoms have existed before energetic antisyphilitic 
treatment was commenced. Often as it remains without effect, 
a trial of it is still indicated if not more than two or four 
months have elapsed after their appearance. If they have 
existed for half a year or longer, nothing can be expected from 
any such treatment, and it need not, therefore, be begun. In 
such cases the prognosis, of course, is worse than in the others. 
Thirdly, a good deal depends upon the kind of symptoms by 
which the infection of the nervous system manifests itself. 
General symptoms, headache, vertigo, epileptiform attacks, 
allow, cceteris paribus, of a more favorable prognosis than focal 
symptoms, such as monoplegias, hemiplegias, and paralyses of 
certain nerves. The worst outlook is afforded by those in- 
stances in which the brain and the spinal cord are equally 
severely attacked, as in tabes and progressive paralysis of the 
insane. 

Treatment. — The manner in which the treatment is to be 
conducted must be made to depend upon the individual case, 
the age, the nutrition of the patient, and so forth, and no rule 
applicable to all cases can be laid down. Only one remark, 
which has repeatedly been made before in this book, we wish 
here again to emphasize, namely, that if we have once decided 
upon adopting the antisyphilitic treatment we must do so 
energetically, giving iodide of potassium, one to six or eight 
grammes (grs. xv-3 jss.-3 ij) daily, in one or two doses in hot 






SYPHILIS OF THE GENERAL NERVOUS SYSTEM. 



703 



milk, continued for from six to ten weeks, and inunctions of 
blue ointment, from three to five grammes (grs. xlv-lxxv) a 
day, continued for from four to six weeks. All necessary pre- 
cautions are self-evident. Finally, we should not neglect to 
familiarize ourselves with the progress which has been made 
in the modern treatment of syphilis, and consider whether the 
subcutaneous use of mercury is advisable, and, if so, what the 
exact mode of its administration should be. 

LITERATURE. 

Oppenheim. Zur Kenntniss der syphilit. Erkrankungen des Centralnerven- 

systems. Berliner klin. Wochenschr., 1889, 48. 
Ziemssen. Syphilis des Nervensystems. Klin. Vortr., iv, November 3, 1888. 
Warrer. British Med. Journ., September, 1888. (Two Cases of Brain-Syphilis 

in One Family.) 
Jiirgens. Berliner klin. Wochenschr., 1888, xxv, 22. (Syphilis of the Spinal 

Cord.) 
Oppenheim. Berliner klin. Wochenschr., 1888, 53. 
Siemerling. Arch. f. Psych, u. Nervenkh., 1888, xx, 1. (Congenital Syphilis of 

the Brain and Spinal Cord.) 
Naunyn. Mittheilungen aus der medicinischen Klinik zu Konigsberg. Leipzig, 

Vogel, 1888. 
Siemerling. Arch. f. Psych., 1890, 1, 2. 
Naunyn. Die Prognose der syphilit. Erkrankungen des Nervensystems, XIII. 

Wanderversammlung der siidwestdeutschen Neurologen. Arch. f. Psych. 

u. Nervenkh., 1889, xx, 2. 
Sanger. Zur Kenntniss der Nervenerkrankungen in der Fruhperiode der Syphi- 
lis. Jahrb. d. Hamburger Staatskrankenanstalten, II. Jahrg., 1890. 
Sachs, B. New York Med. Journ., Sept. 19, 1891. 
Hutchinson, Jr. Syphilitic Disease of the Occipital Lobe with Perforation of 

Cranium. British Med. Journ., March 21, 1891. 
Joffroy et Letienne. Contribution a l'etude de la syphilis cerebrale. Arch, de 

med. experim. et d'anat. pathol., 1891, 3. 
Schulz, R. Neurol. Centralbl., 1891, 19. 
Goldflam. Riickenmarksyphilis. Wiener Klinik, 2, 93. 
Gowers. Syphilis und Nervensystem. German by Lehfeldt, Berlin, Karger, 

1893. 
Cnopf (Niirnberg). Munch, med. Wochenschr., 1892, 11. (Case of Cerebral 

Syphilis.) 
Pick. Zur Kenntniss der cerebro-spinalen Syphilis. Zeitschr. f. Heilkunde, 

1892, 4, 5. 
Nonne. Beitrage zur Kenntniss der syphilit. Erkrankungen des Riickenmarks. 

Hamburg, 1892. 
Obermeier. Deutsche Zeitschr. f. Nervenhk., 1892, iii, 1-3. (Two Cases of 

Gummatous Meningitis.) 
Gaykiewicz (Warsaw). Syphilis du systeme nerveux. Paris, Bailliere, 1892. 
Kowalewsky. Arch. f. Dermat. u. Syph., 1893. 



INDEX 



Abducens, anatomy of, 44 ; paralysis of, 
49 ; paralysis of, in tabes, 632 ; paralysis 
of, in brain tumor, 298. 

Abductor, paralysis of, 113 ; paralysis of, 
in tabes, 636. 

Abscess, of the brain, 260 ; of the spinal 
cord, 465. 

Accessorius, anatomy of, 136 ; lesions of, 
in tabes, 636 ; paralysis of, 138 ; spasm 
of, 137. 

Accommodation, errors of, as a cause of 
headache, 65. 

Acromegaly, 512. 

Adams-Stokes disease, 130. 

Adductor spasm, 114. 

Ageusia, 108. 

Agraphia, 176. 

Agrypnia, 510. 

Akinesia algera, 464. 

Alalia, 153. 

Alcoholic neuritis, 392. 

Alcoholism, treatment of, by suggestion, 
611. 

Alexia, 176. 

Amaurosis in brain tumor, 296. 

Amaurosis, epileptiform, 32. 

Amaurosis, epileptiform, in brain tumor, 
297. 

Amaurosis partialis fugax, 38. 

Amblyopia, alcoholic, 39 ; lead, 39 ; to- 
bacco, 39. 

Amblyopia, central, 32. 

Amyotrophic lateral sclerosis, 447. 

Anaemia, cerebral, 254 ; spinal, 462. 

Anaesthesia of bladder, 378. 

Anaesthesia, doll's head, 562 ; gustatoria, 

108 ; hysterical, 551, 557 ; laryngeal, 

115 ; in transverse myelitis, 45 1 ; in tabes, 

646 ; in traumatic neuroses, 562 ; of 

45 



trigeminus, 73 ; in unilateral cord lesions, 
456. 

Analgesia, in syringomyelia, 472 ; in tabes, 
647. 

Anarthria, 153. 

Aneurisms, of cerebral arteries, 253 ; mil- 
iary, 214 ; of spinal arteries, 462. 

Angina pectoris, 123. 

Angio-neurotic oedema, 133, 399. 

Angiomata of spinal cord, 468. 

Anidrosis, 399. 

Anisocoria, 48 ; in general paralysis, 691 ; 
in neurasthenia, 532 ; in tabes, 632. 

Ankle clonus, 422. 

Antipyrine as a cause of epilepsy, 573. 

Anosmia, 26. 

Ape-hand, 435. 

Aphasia, amnesic, 177 ; in children, 181 ; 
conduction, 176, 179 ; diagnosis of, 177 ; 
Grashey's, 181 ; motor, 176, 179 ; total, 
176 ; reflex, 182 ; sensory, 176, 179. 

Aphonia, hysterical, 542. 

Apoplectic, equivalents, 221 ; stroke, 218. 

Apoplectiform attacks, in general paraly- 
sis, 693 ; in hematoma durae matris, 6. 

Apoplexie foudroyante, 219. 

Apoplexies, capillary, 214. 

Apoplexy, cerebral, 213 ; hysterical, 545 ; 
mental condition in, 228 ; progressive, 
6 ; sensibility in, 224, 228 ; spinal, 45S ; 
spinal-meningeal, 326 ; trophic changes 
in, 231. 

Arachnoid, anatomy of, 3. 

Ai"an-Duchenne type of progressive mus- 
cular atrophy, 434. 

Argyll Robertson pupil, 48 ; in tabes, see 
loss of light reflex, 632, 669. 

Arsenic, as a cause of multiple neuritis, 

389. 

705 



706 



%. 



DEX. 



Arrhythmia cordis, 130. 

Arteries, cerebral, 209 ; dilatation of, 253 ; 
embolism and thrombosis of, 244 ; neu- 
roses of, 254. 

Arteries, spinal, 458 ; dilatation of, 462 ; 
embolism and thrombosis of, 460 ; neu- 
roses of 462. 

Artery of cerebral haemorrhage, 212. 

Arthropathy in tabes, 657. 

Aspermatism, 531. 

Asphyxia, local, 401. 

Aspiration pneumonia in bulbar paralysis, 
156. 

Associated movements, 229. 

Associated movements in tabes, 644. 

Astasia abasia, 548. 

Asthma, bronchial, 118; cardiac, 129; 
hysterical, 120, 543 ; saturnine, 121 ; 
thy mi cum, 114. 

Ataxia, cortical, 186 ; in diabetes, 670 ; 
after diphtheria, 669 ; functional, 669 ; 
hereditary, 442 ; locomotor, 629 ; after 
pregnancy, 670 ; in tabes, 640. 

Athetoid movements in tabes, 644. 

Athetosis, 284 ; bilateral, 259. 

Atrophy, muscular, congenital, 413 ; in 
hemiplegia, 232 ; hysterical, 546 ; myo- 
pathic, 406. 

Atrophy, muscular, progressive spinal, 434. 

Atrophy, optic, 33 ; congenital, 34 ; in 
general paralysis, 693 ; in multiple 
sclerosis, 620 ; in tabes, 630. 

Attack, apoplectic, 218 ; apoplectiform, in 
general paralysis, 693 ; apoplectiform, 
in hoematoma durae matris, 6 ; epileptic, 
575 ; hysterical 553 ; hystero-epileptic, 
600. 

Auditory nerve, anatomy of, 95 ; hyper- 
esthesia of, 97 ; paralysis of, 97 ; paraly- 
sis, rheumatic, 97 ; in tabes, C63. 

Aura, epileptic, 575. 

Automatism in hypnotism, 603. 

Basal ganglia, anatomy of, 198 ; lesions 

of, 198. 
Basedow's disease, 518. 
Bedsore, acute, malignant, 232. 
Beri beri, 331. 
Birth palsy, 354. 
Bladder, anaesthesia of, 378 ; disturbances 

of, in myelitis, 452 ; disturbances of, in 

tabes, 652. 
Blepharoptosis cerebralis, 46. 



Blepharospasm, 79. 

Brachial plexus, anatomy of, 334 ; diseases 

of, 340. 
Bradycardia, 129. 
Brain abscess, 260 ; aetiology of, 261 ; 

diagnosis of, 264 ; prognosis of, 265 ; 

symptoms of, 262. 
Brain syphilis, 700. 
Brain tumor, 2S9 ; aetiology of, 292, 

choked disc in, 296 ; diagnosis of, 299 ; 

epileptiform convulsions in, 294 ; focal 

symptoms in, 297 ; mental changes in, 

294 ; nature of, 302 ; seat of, 301 ; 

symptoms of, 292. 
Breast, irritable, 365. 
Bromides in epilepsy, 590. 
Brown-Sequard's paralysis, 456. 
Bulbar paralysis, 152. 
Burdach's columns, 420. 

Cachexie exophthalmique, 518. 

Cachexie pachydermique, 525. 

Cadaveric position of vocal cords, 116. 

Capsule, internal, anatomy of, 190 ; le- 
sions of, 199. 

Carcinoma of brain, 290 ; of cord, 468 ; of 
vertebrae, 453. 

Cardiac branches of vagus, 123. 

Cardialgia, 131. 

Caries of the spine, diseases of the cord in, 
453 ; pachymeningitis spinalis due to, 
316. 

Catalepsy, 602. 

Cauda equina, tumors of, 469. 

Cavity formation in cord, 471. 

Centrum ovale, 189. 

Centrum ovale, lesions of, 195. 

Cephalalgia, cf. Headache. 

Cephalocele, 312. 

Cerebellar, abscess, 263, 265 ; ataxia, cf. 
Equilibrium, loss of, in diseases of the 
cerebellum ; peduncles, lesions of, 206 ; 
tract, direct, 420. 

Cerebellum, lesions of, 205. 

Cerebral lesions, pathological diagnosis of, 
209 ; topical diagnosis of, 162. 

Cerebral palsy of children, 268. 

Charcot's joint, 657. 

Cheyne-Stokes breathing in apoplexy, 
220. 

Chiasm, optic, anatomy of, 29 ; lesions of, 

34- 
Choked disc, cf. Papillitis. 



IN. 



DEX. 



707 



Cholesteatoma of brain, 290. 

Chorda tympani, lesions of, 88. 

Chorea, 481 ; congenital, 488 ; hereditary, 
488 ; Huntington's, 488 ; imitatoria, 
488 ; major and minor, 481 ; in preg- 
nancy, 4S8 ; senile, 488 ; Sydenham's, 
481. 

Circle of Willis, 210. 

Circumflex nerve, paralysis of, 353. 

Claw-hand, 352, 435. 

Clonus, ankle, 422. 

Clovvnism, 601. 

Club foot, paralytic, 428. 

Cocaine as a cause of epilepsy, 573. 

Coccygeal nerves, anatomy of, 370. 

Coccygodynia, 380. 

Cold-water treatment in hysteria, 568. 

Color vision, disturbances of, in papillitis, 
33 ; in tabes, 631 ; in hysteria, 541. 

Coma, in cerebral haemorrhage, 220 ; dif- 
ferential diagnosis of, 233 ; epileptic, 
233 ; in pachymeningitis interna haemor- 
rhagica, 6. 

Combined system disease, 442. 

Compression myelitis, 453 

Concussion, spinal, cf. Traumatic neuro- 
ses. 

Conjugate deviation of eyes, 222. 

Contractures in infantile cerebral palsies, 
272 ; in spinal paralysis, 428 ; in hemi- 
plegia, 226; in hysteria, 550, 556. 

Convergence, insufficiency of, in Graves' 
disease, 520. 

Convolutions of the brain, 167. 

Convulsions, epileptiform, in brain tumor, 
299 ; in cerebral palsies of children, 
271 ; in general paralysis, 693. 

Convulsions, hystero-epileptic, 600 ; infan- 
tile, cf. Eclampsia ; puerperal, 595 ; in 
spinal paralysis of children, 427. 

Co-ordination, disturbances of, in tabes, 
640. 

Cornu Ammonis in epilepsy, 587. 

Corpora albicantia, 203. 

Corpora quadrigemina, 199 ; lesions of, 200. 

Corpus callosum, absence of, 313. 

Cortical, ataxia, 186 ; epilepsy, 186 ; motor 
disturbances, 183 ; sensory disturbances, 
188. 

Cough, hysterical, 543. 

Cough, trigeminal, 76. 

Cranial nerves, diseases of, 24 ; in apo- 
plexy, 223 ; in brain tumor, 298 ; in 



hysteria, 541 ; in tabes, 630 ; multiple 
affections of, 147. 

Cremasteric reflex, 421. 

Crises, anal, in tabes, 635 ; crises, gas- 
tric, 634; laryngeal, 636; pharyngeal, 

634. 
Crura cerebri, 202. 
Crutch palsy, 345. 
Crying fits in hysteria, 543. 
Cyst, apoplectic, 214. 
Cysticercus, of brain, 305 ; of spinal cord, 

470. 

Dairymaid's cramp, 357. 

Deafness, in hysteria, 541 ; in meningitis. 
20; in tabes, 634; word, 176. 

Degeneration, of nerves, 332 ; reaction of, 
91 ; secondary, 445 ; signs of, in epilep- 
tics, 584. 

Deglutition paralysis, hysterical, 544. 

Dementia in brain tumor, 295. 

Dementia paralytica, 688. 

Depression in general paralysis, 692. 

Deviation, conjugate, 51. 

Deviation of eyes, primary, 51 ; secondary, 

51. 

Diabetes, knee-jerk in, 650; multiple neu- 
ritis following, 388 ; ataxia following, 
670. 

Diaphragm, paralysis of, 336 ; spasm of, 

336- 

Diarrhoea, in tabes, 635. 

Diffuse sclerosis, cerebral, 267. 

Diffusion electrode, 72. 

Digestive disturbances in affections of the 
vagus, 130. 

Diphtheria, ataxia following, 669 ; hemi- 
plegia following, 217 ; laryngeal paraly- 
sis following, 113 ; laryngeal anaesthesia 
following, 115 ; pharyngeal paralysis 
following, 149 ; neuritis following, 388 ; 
oculo-motor paralysis following, 46. 

Diplegia, cerebral, 282 ; facial, 86. 

Diplopia, crossed, 51 ; homonymous, 51 ; 
monocular, 46 ; in tabes, 632. 

Dorsal nerves, 363. 

Double images, examination for, 51. 

Double vision, cf. Diplopia. 

Duchenne's disease, 152. 

Dura mater, cerebral, 3 ; spinal, 316. 

Dyspepsia, nervous, 133. 

Dysphagia, spasmodic, 134. 

Dystrophy, progressive muscular, 406. 



7o8 



INDEX. 



Ear disease, in brain abscess, 261 ; and 

meningitis, 10. 
Ecchymoses, cutaneous, in tabes, 645. 
Echinococcus, of brain, 307 ; of spinal 

cord, 470. 
Eclampsia, 594. 

Embolism, cerebral, 244 ; spinal, 460. 
Encephalitis, non-suppurative, 266 ; puru- 
lent, 260. 
Encephalocele, 312. 
Encephalomalacia, 244. 
Encephalopathy, saturnine, 255. 
Enchondroma of brain, 290. 
Endarteritis, cerebralis syphilitica, 252 ; 

spinalis syphilitica, 461. 
Endocarditis and chorea, 485. 
Enuresis nocturna, 379. 
Epilepsia, acetonica, 586 ; procursiva, 582. 
Epilepsy, cortical, 186 ; and heart disease, 

586 ; Jacksonian, 186 ; reflex, 574 ; 

saturnine, 587 ; and syphilis, 572 ; trau- 
matic, 573. 
Epileptic, absence, 581 ; attack, 575 ; aura, 

575 ; equivalents, 582, insanity, 582 ; 

vertigo, 581. 
Erector spina?, paralysis of, 365. 
Erythema nodosum, 399. 
Erythromelalgia, 398. 
Ether, subcutaneous injection of, as a cause 

of musculospiral paralysis, 347. 
Equilibrium, loss of, in diseases of the 

cerebellum, 205. 
Equivalent, apoplectic, 221 ; epileptic, 

582 ; hemicranic, 509. 
Exophthalmic goitre, 518. 
Eye muscles, paralysis of, 49 ; in brain 

tumor, 29S ; in mei^inigitis, 12, 16 ; in 

tabes, 032. 

Facial nerve, anatomy of, 77 ; in tabes, 633. 

Facial paralysis, bulbar, 84 ; electrical re- 
actions in, 91 ; movements of expression 
in, 83 ; peripheral, 87 ; pontine, 84, 85 ; 
prognosis in, 91 ; rheumatic, 89. 

Facial spasm, 78. 

Facio-lingual monoplegia, 84. 

Feeding system (Weir Mitchell), 567. 

Fifth nerve, cf. Trigeminus. 

Fissures of brain, 168. 

Flexibilitas cerea, 603. 

Flexor contractures in meningitis, 14. 

Foot-clonus, cf. Clonus. 

Forced, movements, 206 ; positions, 206. 



Formes frustes, in Graves' disease, 522 ; 
in multiple sclerosis, 620 ; in tabes, 666. 
Fothergill's faceache, 68. 
Fourth nerve, cf. Patheticus. 
Fractures in tabes, 656. 
Friedreich's disease, 442. 
Front tap, 422. 

Gait, in multiple neuritis, 392 ; in pseudo- 
hypertrophy, 365 ; in spastic paralysis, 
440 ; in tabes, 640. 

Gangrene, symmetrical. 401. 

Gasserian ganglion, 56. 

Gastralgia, 131. 

Gastric branches of vagus, 130. 

Gastric crises, 634 ; vertigo, 101. 

Gastrodynia, 132. 

General paralysis, 688 ; aetiology of, 688 ; 
pathological anatomy of, 694 ; and tabes, 
638 ; treatment of, 697. 

Gerlier's disease, 101. 

Giddiness, cf. Vertigo. 

Gilles de la Tourette's disease, cf. Maladie 
des tics convulsifs. 

Girdle sensation in tabes, 645. 

Glioma of the brain, 289 ; of the spinal 
cord, 467. 

Gliosis in syringomyelia, 471. 

Globus hystericus, 544. 

Glossopharyngeal nerve, anatomy of, 107 ; 
lesions of, 108. 

" Glossy skin," 400. 

Glottis, spasm of, 114 ; hysterical, 542. 

Gluteal reflex, 421. 

Goitre, exophthalmic, 518. 

Goll's columns, 420. 

Graefe's symptom in Graves' disease, 519. 

Grand mai, 581. 

Graphospasm, 356. 

Graves' disease, 518. 

Gray matter of spinal cord, diseases of, 425. 

Gubler's tumor, cf. Tenosynovitis. 
I Gyri, cerebral, 167. 

Heematoma durse matris, 4. 
Haematomyelia, 458. 

Hasmatorrhachis, cf. Meningeal hemor- 
rhage, 326. 
Hematuria in tabes, 653. 
Haemorrhage, cerebral, 213 ; spinal, 458 ; 

spinal-meningeal, 326. 
Hair, falling out of, in Graves' disease, 521. 
I Hallucinations in hysteria, 541. 



INDEX. 



yog 



Headache, aetiology of, 63 ; anatomical 
seat of, 61 ; in brain syphilis, 702 ; in 
brain tumor, 293 ; in pachymeningitis, 
6 ; treatment of, 65. 

Hearing, disturbances of, cf. also Auditory 
nerve, 95 ; in engineers, 98 ; in facial 
paralysis, S8. 

Heart, affections of, in vagus lesions, 123 ; 
in Graves' disease, 518. 

Heart disease and chorea, 485 ; and em- 
bolism, 244. 

Hemianesthesia, in diseases of the inter- 
nal capsule, 199, 227 ; hysterical, 545 ; 
in syringomyelia, 472. 

Hemianopia, 34 ; in cerebral syphilis, 36 ; 
examination for, 36 ; oscillating, 36. 

Hemianopic pupillary reaction, 35. 

Hemiataxia, 229. 

Hemiathetosis, 286, 

Hemiatrophia, facialis, 403 ; lingualis, 

144, 637- 

Hemichorea, 481 ; posthemiplegic, 228 ; 
prehemiplegic, 218. 

Hemicrania, 507. 

Hemiopia, 34. 

Hemiplegia, 224; alternating, 85, 204; 
cerebral, 224 ; direct, 225 ; in general 
paralysis, 693 ; hysterical, 545 ; indirect, 
225 ; infantile spastic, 271 ; post-diph- 
theritic, 217 ; spinal, 457 ; in tabes, 
638. 

Hemiparesis, 224. 

Hemisection of the spinal cord, 456. 

Hemispasm, glosso-labial, 81. 

Hepatalgia, 132. 

Hereditary ataxia, 442. 

Hereditary chorea, 4S8. 

Herpes, labialis, in meningitis, 18 ; zoster, 
364, 400. 

Hip muscles, spasm of, 385. 

Huntington's chorea, 488. 

Hydrarthrosis, 400. 

Hydrocephalus, 308. 

Hydromyelia, 471. 

Hydrorrhachis, 471. 

Hyperacusis, in facial paralysis, 89. 

Hyperemia, cerebral, 254; spinal, 462. 

Hyperesthesia, of auditory nerve, 96 ; in 
Brown-Sequard's paralysis, 457 ; hys- 
terical, 551, 557; laryngeal, 115; in 
meningitis, 12 ; plantar, 377 ; in tabes, 

645- 
Hyperemesis nervosa, 134. 



Hyperidrosis, 399 ; of the face, 69 ; in 
facial paralysis, 90. 

Hyperkinesis cordis, 126. 

Hypertrophy of muscles, 412. 

Hypnotism, 602. 

Hypoglossal nerve, anatomy of, 140 ; in 
hemiplegia, 223 ; paralysis of, 141 ; 
spasm of, 144 ; in tabes, 637. 

Hysteria, 539. 

Hysteria, aortic, 543 ; in the male, 554. 

Hysterical, aphonia, 542 ; asthma, 543 ; 
convulsions, 553 : hemianesthesia, 545 ; 
hemiplegia, 545; ischuria, 552; neural- 
gia, 551 ; paralysis, 548 ; vomiting, 544. 

Hystero-epilepsy, 600. 

Hysterogenic zones, 551. 

"Idiotie myxcede'mateuse," 526. 
Imitation epilepsy, 574. 
Inequality of pupils, cf. Anisocoria, 48. 
Infantile, cerebral palsy, 268 ; convulsions, 

cf. Eclampsia, 594 ; hemiplegia, 271 ; 

spinal palsy, 426. 
Infectious diseases and meningitis, 11. 
Infraorbital neuralgia, 69. 
Insanity, post-epileptic, 578 ; pre-epilep- 

tic, 575- 
Insomnia, treatment of, 510. 
Insula, cf. Island of Reil. 
Insular sclerosis, cf. Multiple sclerosis. 
Intention tremor, 618. 
Intercostal neuralgia, 363. 
Internal capsule, 190 ; lesions of, 199. 
Internal popliteal nerve, 381. 
Involuntary movements in tabes, 643, 644. 
Irritation, spinal, 463. 
Island of Reil, 171. 
Ischuria, hysterical, 552. 

Jacksonian epilepsy, 186. 
Jaw-jerk, 447. 

Joint, Charcot's, 657 ; hysterical, 551. 
Juvenile muscular atrophy, 408. 

Kak-ke, 331. 

Kakosmia, 26. 

Knee-jerk, 422 ; centre for, 422 ; in 
chronic alcoholism, 650 ; in diabetes, 
650 ; in hemiplegia, 227,231 ; in heredi- 
tary ataxia, 441 ; in multiple sclerosis, 
619 ; in neuritis, 392, 650 ; in tabes, 
650. 

Kyphosis, 365. 



yio 



INDEX. 



Labio-glosso-laryngeal paralysis, 152. 

Lachrymation, transient, in tabes, 632. 

Lancinating pains in tabes, 645. 

Landry's paralysis, 466. 

Laryngeal anaesthesia, 115. 

Laryngeal ataxia in tabes, 636 ; hyper- 
esthesia, 115. 

Laryngeal muscles, paralysis of, 113, 116 ; 
spasm of, 114. 

Laryngismus stridulus, 114. 

Lateral sclerosis, 440. 

Lateral sclerosis, amyotrophic, 447. 

Laughing fits in hysteria, 543. 

Lead palsy, 347. 

Lenticulo-optic artery, 212. 

Lenticulo-striate artery, 212. 

Leptomeningitis, cerebral, 8 ; spinal, 322. 

Lethargy in hypnotism, 603. 

Leucomyelitis, 439. 

Levator palpebral, paralysis of, cf. Ptosis. 

Lightning, neuroses caused by, 565. 

Lipoma of brain, 290. 

Localization, cerebral, 163, 165, 171 ; of 
spinal-cord lesions, 423. 

Locomotor ataxia, 629. 

Lumbago, 415. 

Lumbar cord, lesions of, 425. 

Lumbar nerves, 366. 

Lumbo-abdominal neuralgia, 369. 

Mai perforant du pied, 656. 

Maladie des tics convulsifs, 549. 

Malum Cotunnii, 371. 

Maniacal exaltation in general paralysis, 
692. 

Mankopf s symptom, 563. 

Massage in hysteria, 56S. 

Mastication, paralysis of muscles of, 59. 

Mastication, spasm of muscles of, 58. 

Mastodynia, 365. 

Mastoid disease and meningitis, 10. 

Median nerve. 349 ; paralysis of, 350 ; 
sensory affections of, 352. 

Medulla oblongata, 206 ; lesions of, 207. 

Meniere's disease. 99, 103. 

Meningitis, epidemic, symptoms of, 13 ; 
diagnosis of, 18. 

Meningitis, gummatous, 9 ; idiopathic 
purulent, 12; pseudo-, hysterical, 19; 
serous, 18 ; tuberculous, 10 ; in adults, 
16 ; tuberculous, in children, 15 ; spi- 
nal, 322. 

Mercurial tremor, C24. 



Merycism, 131. 

Mesmerism, 604. 

Metatarsalgia, 377. 

Meteorism, 544. 

Meteorological condition and meningitis, 
11. 

Middle cerebral artery, 211. 

Migraine, 507 ; ophthalmic, 508. 

Miliary aneurisms, 214. 

Mimic facial spasm, 78. 

Mind-blindness, 176. 

Mind-deafness, cf. Word-deafness, 176. 

Miners' nystagmus, 53. 

Mogigraphia, 356. 

Monocontracture, 186. 

Monoparesis, 185. 

Monoplegia, cortical, 185 ; facial, 84. 

Monoplegia, facio-lingual, 84. 

Motor centres, 184. 

Motor disturbances in Graves' disease, 521. 

Motor-oculi, cf. Oculo-motcrius, 42. 

Motor points, of arm, 347, 350, 352 ; of 
face and neck, 93, 355 ; of leg, 382, 383. 

Multiple neuritis, 387. 

Multiple sclerosis, 616. 

Muscular atrophy, juvenile, 4C8 ; progres- 
sive, 434. 

Muscular rheumatism, 414. 

Muscular sense in tabes, 642. 

Musculo-cutaneous nerve, lesions of, 353. 

Musculo-spiral nerve, paralysis of, 344 ; 
in lead poisoning, 347 ; paralysis due to 
subcutaneous injection of ether, 347. 

Musculo-spiral nerve, spasm of, 348. 

Mutism, hysterical, 543. 

Myalgia, 414. 

Myelitis, acute, 465 ; cervical, 424 ; chron- 
ic, 467 ; dorsal, 424 ; lumbar, 425 ; 
purulent, 465 ; transverse, 450 ; trans- 
verse syphilitic, 441. 

Myelomalacia, 460. 

Myoclonia congenita, 549. 

Myoclonus multiplex, 549. 

Myopathy, progressive, atrophic, 41 1. 

Myosis, spinal, 48 ; in tabes, 632. 

Myotonia congenita, 497. 

Myx oedema, 525. 

Nails, falling out of, in tabes, 655. 
Nasal disease and asthma, 119. 
Nerve-stretching in tabes, 684. 
Neuralgia, cervico-occipital, 338 ; cceliaca, 
132 ; crural, 369 ; of external genitals, 



IXDEX. 



711 



377 ; hysterical, 557 ; infra-orbital, 69 ; 
intercostal, 363 ; lurnbo-abdominal, 369 ; 
of prostate, 378 ; spermatic, 369 ; supra- 
orbital, 69 ; trigeminal, 6S ; of urethra, 
37 3. 

Neurasthenia, 529. 

Neuritis, 331 ; due to alcohol, 392 ; due 
to arsenic, 389 ; infectious, 388 ; migrans, 
331 ; nodosa, 331 ; multiple, 387 ; mul- 
tiple, mental symptoms in, 390 ; multiple, 
as a sequence of other diseases, 3S8 ; 
optic, cf. Papillitis ; purulent, 331 ; 
retro-bulbar, 32. 

Neuro-fibroma plexiforme, 332. 

Neuroma, 332. 

Neuroses, caused by lightning, 565 ; 
traumatic, 561. 

Nicotine poisoning, 128. 

Night palsy, 400. 

Nystagmus, 53 ; in Friedreich's disease, 
442 ; in multiple sclerosis, 618 ; oscilla- 
torius, 53. 

Obturator nerve, paralysis of, 369. 

Occipital, lobe, lesions of, 172, 176 ; neu- 
ralgia, 338. 

Ocular vertigo, 48, 100. 

Oculo-motor nerve, anatomy of, 42. 

Oculo-motor paralysis, central, 46 ; com- 
plete, 47 ; cortical, 46 ; peripheral, 
45 ; rheumatic, 45. 

Occupation neuroses, 356. 

CEdema, angio-neurotic, 133, 399. 

OZsophagismus, 134. 

OZsophagus, spasm of, 134. 

Olfactory, centre, 25 ; nerve, anatomy of, 
25 ; nerve, hyperesthesia of, 27 ; nerve, 
central lesions of, 27 ; nerve, peripheral 
lesions of, 27 ; nerve, in tabes, 630. 

Ophthalmia paralytica, 74. 

Ophthalmoplegia, externa, 50 ; interna, 50 ; 
progressiva, 151. 

Optic, atrophy, cf. Atrophy, optic, 33 ; cen- 
tre, 30 ; chiasm, 29, 34 ; nerve, anat- 
omy of, 29 ; nerve, diseases of, 30 ; 
neuritis, cf. Papillitis ; neuritis, retro- 
bulbar, 32 ; radiation, anatomy of, 30 ; 
radiation, lesions of, 35 ; thalamus, lesions 
of, 198 ; tract, 34. 

Osteo-sarcoma of brain, 290. 

Osteo-arthropathy, 516. 

Otitis media, as a cause of brain abscess, 
261 ; as a cause of meningitis, 10. 



Ovarian hyperesthesia, 551. 
Oxyacoia, 97. 

Pachymeningitis, cerebral, 4 ; cervicalis 
hypertrophica, 317 ; interna hsemor- 
rhagica, 4 ; spinalis, 316. 

Pains, lancinating, in tabes, 645. 

Palate, innervation of, 148. 

Palpitation of the heart, 126. 

Palsy, combined shoulder-arm, 35 5 ; night, 
400. 

Papillitis, 30 ; in brain abscess, 262 ; in 
brain tumor, 31 ; in meningitis, 12 ; in 
neuropathic individuals, 33. 

Papillo-retinitis, 32. 

Paracusis Willisii, 98. 

Paradoxical contraction, 423. 

Paresthesia in tabes, 646. 

Parageusia, 109. 

Paralysis, acute ascending, 466 ; agitans, 
500 ; Brown-Sequard's, 456 ; bulbar, 
152 ; cortical, 185 ; glosso-labio-laryn- 
geal, 152 ; glosso-labio-pharyngea cere- 
bralis, 250 ; hysterical, 548 ; infantile 
spinal, 426 ; Landry's, 466 ; post-diph- 
theritic, 14S ; pseudo-bulbar, 250 ; 
pseudo-hypertrophic, 412; spastic spi- 
nal, 441. 

Paramyoclonus multiplex, 549. 

Paraplegia, ataxic, 443. 

Parasites of brain, 305. 

Parasites of cord, 470. 

Parietal lobes, 168. 

Parkinson's disease, 500. 

Patellar reflex, cf. Knee-jerk. 

Patheticus, anatomy of, 43 ; lesions of, 49. 

Peduncles, of cerebellum, 206 ; of cere- 
brum, 202. 

Perforating ulcer of the foot, 656. 

Periarteritis, 213. 

Perimetric examination, 36. 

Peroneal paralysis, cf. Popliteal nerve, 
external, paralysis of. 

Petit Mai, 581. 

Pharynx, paralysis of, 14S. 

Phonic paralysis, 116. 

Phrenic nerve, neuralgia of, 337 ; paraly- 
sis of, 336 ; spasm of, 336. 

Pia mater, cerebral, 3 ; spinal, 315. 

Pineal gland, tumor of, 297. 

Pitres-Nothnagel sections, 194. 

Pituitary body, tumor of, 297, 29S. 

Plaques jaunes, 246. 



712 



INDEX. 



Plexus, brachial, lesions of, 340. 

Plexus, cervical, anatomy of, 332 ; lesions 
of, 336. 

Plexus, lumbar, lesions, 366. 

Plexus, sacral, lesions of, 370. 

Pneumogastric nerve, cf. Vagus. 

Points, tender, in intercostal neuralgia, 
364 ; in sciatica, 372 ; in trigeminal 
neuralgia, 68. 

Polio-encephalitis (Strumpell), 268 ; (Wer- 
nicke), 150. 

Poliomyelitis, anterior acuta, 426 ; chron- 
ica, 432. 

Polyaesthesia in tabes, 647. 

Polydipsia in brain syphilis, 700. 

Polyneuritis, cf Multiple neuritis, 387. 

Polyuria, in brain syphilis, 700 ; in hys- 
teria, 552 ; in meningitis, 14. 

Pons, 204. 

Popliteal nerve, external, paralysis of, 381. 

Popliteal nerve, internal, paralysis of, 
381. 

Porencephaly, 267, 312. 

Post-diphtheritic paralysis, 148. 

Post-epileptic phenomena. 578. 

Posterior fossa, tumor of, 299. 

Pott's disease, 453. 

Pi-e-epileptic insanity, 575. 

Pregnancy, ataxia following, 670. 

Pressure myelitis, 453. 

Professional spasms, 356. 

Progressive, bulbar paralysis, 152 ; muscu- 
lar atrophy, 434; ophthalmoplegia, 151 ; 
paralysis of the insane, 688. 

Prosopalgia, 68. 

Propulsion, 504. 

Psammoma of brain, 290. 

Pseudo-apoplexy, 221. 

Pseudo-bulbar paralysis, 158, 250. 

Pseudo-hypertrophy of muscles, 366, 412. 

Pseudo-meningitis, hysterical, 19. 

Pseudo-tabes peripherica, 388. 

Psychical blindness, 176 ; deafness, 176 ; 
condition, after apoplexy, 228 ; in brain 
tumor, 295. 

Psychoses in tabes, 638. 

Ptosis, 47 ; in tabes, 632. 

Puerperal, convulsions, 594 ; eclampsia, 

594- 
Pulmonary branches of vagus, 118. 
Pulse, in brain tumor, 295 ; in meningitis, 

12 ; slowing of, in lesions of cervical 

cord, 424. 



Pulvinar, 30, 35, 198. 
Pupil, Argyll Robertson, 48. 
Pupil, inequality of, cf. Anisocoria. 
Pupillary reaction, 48 ; hemianopic, 35. 
Pyramidal tract, anatomy of, 420. 

Quadratus lumborum, spasm of, 385. 
Quadrigeminal bodies, anatomy of, 199 ; 

lesions of, 200. 
Quinine as a cause of amblyopia, 40. 
Quinine in Meniere's disease, 105. 

Radial paralysis, cf. Musculo-spiral paral- 
ysis, 344. 

Railway spine, 561. 

Raynaud's disease, 401. 

Reaction of degeneration, 91. 

Rectal symptoms in tabes, 652. 

Rectum, centre for, 423. 

Recurrent laiyngeal paralysis, 113. 

Reflex, abdominal, 421 ; arc, 421 ; cre- 
masteric, 421 ; gluteal, 421 ; patellar, 
cf. Knee-jerk, 421; periosteal, 447; 
plantar, 421 ; pupillary, cf. Pupillary 
reaction ; tendo Achilles, 422. 

Reflexes, deep and superficial, 421 ; in 
apoplexy, 225, 227 ; in epilepsy, 547. 

Relation of cortex to skull, 169. 

Respiratory organs, diseases of, in vagus 
affections, 118 ; nerves of, 118. 

Rest cure, 567. 

Retropulsion, 504. 

Rheumatic, acusticus paralysis, 97 ; oculo- 
motor paralysis, 45 ; facial paralysis, 
89. 

Rheumatism, and chorea, 484 ; muscular, 
414. 

Rickets and laryngismus stridulus, 1 14. 

Rigidity of muscles in paralysis agitans, 
502. 

Rigidity of neck in meningitis, 12. 

Rinne's test, 104, 

Romberg's sign, 641. 

Root zone, 420, 649. 

Ructus hystericus, 544. 

Sacral nerves, 370. 

Salivary secretion in facial paralysis, 86 ; 

in bulbar paralysis, 155. 
Saltatory spasm, 386. 
Sarcoma, of brain, 290 ; of cord, 467. 
Scanning speech, 618. 
Scar, apoplectic, 215. 



INDEX. 



7*3 



Scarlet fever, multiple neuritis following, 
38S. 

Sciatica, 371. 

Sciatic nerve, paralysis of, 382. 

Sclerodactyly, 401. 

Scleroderma, 402. 

Sclerose en plaques, 616 

Sclerosis, amyotrophic lateral, 447 ; com- 
bined posterior and lateral, 442 ; diffuse 
cerebral, 267 ; disseminated, 616 ; lateral, 
440 ; lobar, 267 ; multiple, 616; posterior 
spinal, 629. 

Scoliosis, 365. 

Scoliosis in sciatica, 373. 

Scotoma, central, 39 ; flitting, 38 ; simple, 

39- 

Seborrhcea, 399. 

Secretion, anomalies of, in hysteria, 
552. 

Secretion, cutaneous disturbances of, 399. 

Senile, chorea, 488 ; softening, 246 ; tre- 
mor, 506, 623. 

Sensory, aphasia, 176, 179 ; conduction in 
cord, 420 ; cross-way, 199 ; disturbances 
in apoplexy, 227, 228 ; disturbances, cor- 
tical, 188 ; disturbances in neurasthenia, 
530; disturbances in peripheral nerve 
lesions, 334 ; in tabes, 645. 

Serratus, paralysis of, 340. 

Sexual functions, disturbances of, in neu- 
rasthenia, 530 ; in tabes, 654. 

Shaking palsy, 500. 

Shoulder-arm paralysis, 354. 

Shoulder muscles, paralysis of, 138, 139 ; 
spasm of, 138. 

Simulation in epilepsy, 587. 

Singultus, 337 ; in hysteria, 544. 

Sinus thrombosis, 258. 

Sinuses, cerebral, 257. 

Sixth nerve, cf. Patheticus. 

Skin eruptions in meningitis, 14, 18. 

Sleep, hysterical, 540. 

Sleep-palsy, 346. 

Sleeplessness, 510. 

Smell, centre for, 25 ; disturbances of, 26 ; 
examination of, 27. 

Softening, cerebral, cf. Encephalomalacia, 
244 ; multiple foci of, 249 ; spinal, cf. 
Spinal myelomalacia, 460. 

Somnambulism, 602. 

Spasm, bronchial, 118 ;, of glottis, 114 ; 
saltatory, 386. 

Spasmus nictitans, 79. 



Speech, centre for, 179 ; in dementia 
paralytica, 691 ; disturbances of, cf. 
Aphasia ; in Friedreich's disease, 442 ; 
in multiple sclerosis, 618. 

Sphincters, disturbances of, in cord dis- 
eases, 452 ; in tabes, 652. 

Spina bifida, 473. 

Spinal, apoplexy, 458 ; hemiplegia, 457 ; 
irritation, 463 ; leptomeningitis, 322 ; 
muscular atrophy, 434 ; pachymeningitis, 
316 : paralysis, acute ascending, 466 ; 
paralysis, Bro.vn-Sequard's, 456 ; para- 
lysis in children, 426 ; paralysis, spastic, 
441. 

Spinal cord, abscess of, 465 ; anatomy of, 
418 ; blood supply of, 458 ; compression 
of, 453 ; concussion, cf. Traumatic neu- 
roses ; congenital diseases of, 471 ; 
haemorrhage into, 459 ; parasites of, 
470 ; softening of, 460 ; syphilis of, 461 ; 
tumors of, 467. 

Spondylarthrocace, 453. 

Spondylitis, tubercular, 453. 

Status epilepticus, 582. 

Stellwag's symptom, 519. 

Stenocardia, 123. 

Sterno-cleido-mastoid, paralysis of, 138; 
spasm of, 137. 

Strabismus, 47. 

Strise atrophicse, 400. 

Stroke, apoplectic, 218 ; in cerebral em- 
bolism, 247. 

Suggestion, hypnotic, 604. 

Superior oblique muscle, 49. 

Surgical treatment, of abscess, 265 ; of 
tumor, 303. 

Suspension treatment in tabes, 685. 

Sydenham's disease, cf. Chorea. 

Syllable-stumbling, 691. 

Syncope and apoplexy, 233. 

Syphilis, and dementia paralytica, 689 ; of 
the nervous system, 700 ; of the spinal 
cord, 461 ; and tabes, 677. 

Syphilitic basal meningitis, 9. 

Syphiloma of brain, 290. 

Syphiloma of cord, 468. 

Syringomyelia, 471, 472. 

System disease, combined, 442. 

System diseases of the spinal cord, 440. 

Tabes, and alcoholism, 392 ; dorsalis, 629 ; 

pseudo-, 388. 
Tabetic foot, 658. 



714 



INDEX. 



Taches ce'rebrales, 15. 

Tachycardia, 127. 

Tachycardia strumosa exophthalmica, 518. 

Tailor's cramp, 357. 

Talipes, in infantile palsy, cf. Club foot, 

428 ; in external popliteal paralysis, 381. 
Taste, examination of, 108 ; disorders of, 

108 ; disorders of, in facial paralysis, 88, 
Teeth, falling out of, in tabes, 656. 
Tegmentum, 203. 
Telegraphers' cramp, 357. 
Temporal lobe, 169. 
Tendon reflexes, cf. Reflexes, 421. 
Tensor fasciae latae, spasm of, 385. 
Tenosynovitis hypertrophica in wrist- 
drop, 346. 
Terminal arteries, 211. 
Testicle, irritable, 369. 
Tetanilla, 493. 
Tetanus intermittens, 493. 
Tetany, 493. 

Thalamus, optic, 30, 35, 198. 
Third nerve, cf. Oculo-motorius. 
Thomsen's disease, 496. 
Thoracic nerve, anterior, 343 ; posterior, 

340. 
Thrombosis, cerebral, 245 ; sinus, 258 ; 

spinal, 460 ; venous, 258. 
Thyroid gland, in Graves' disease, 518 ; in 

myxcedema, 525. 
Thyroid treatment of myxcedema, 527. 
Tibial nerve, cf. Internal popliteal. 
Tic, convulsif, 78 ; douloureux, 63 ; rota- 

toire, 137. 
Tinnitus aurium, 97. 
Tobacco amblyopia, 39. 
Tongue, atrophy of, 144 ; hemiatrophy of, 

144, 637 ; paralysis of, 144 ; spasm of, 

144. 
Torticollis, 137 ; rheumatic, 414. 
Tracts, of cord, 420 
Trapezius, paralysis of, 138 ; spasm of, 

137- 

Traumatic, neuroses, 561 ; objective symp- 
toms of, 563 ; reaction of muscles, 563. 

Traumatism, in meningitis, 10 ; in tabes, 
676. 

Tremor, alcoholic, 622 ; in Graves' dis- 
ease, 522; in hemiplegia, 230; hyster- 
ical, 557; intention, 618; in multiple 
sclerosis, 618 ; senile, 623 ; in tabes, 643, 
644. 

Trigeminal, cough, 76 ; neuralgia, 68. 



Trigeminus, anatomy of, 56 ; anaesthesia 
of, 73 ; central lesions of, 58 ; extra- 
cranial lesions of, 6S ; intra-cranial 
lesions of, 61 ; peripheral lesions of, 60 ; 
nuclei of, 56 ; paralysis of, 73. 

Trismus, 58. 

Trochlears, cf. Patheticus. 

Trophic disturbances, in apoplexy, 231 ; 
in chorea, 485 ; in hysteria, 552 ; in 
syringomyelia, 472 ; in tabes, 655. 

Trophic nerves, 397 ; general affections 
in which they are chiefly implicated, 
512. 

Trousseau's sign in tetany, 493. 

Tubercle, of brain, 290 ; of cord, 468. 

Tubercular disease of the spine, 453. 

Tubercular meningitis, 9. 

Tumor, cerebral, 2S9 ; spinal, 467 ; of 
spinal meningeal, 328. 

Twitchings, fibrillary, in chronic muscular 
atrophy, 437 ; in neurasthenia, 533. 

Ulcer, perforating, 656. 
Ulnar nerve. 349 ; paralysis of, 351 ; sen- 
sory affections of, 352. 
Upper-arm type of palsy, 431. 
Uraemia and apoplexy, 234. 

Vagus, anatomy of, no ; lesions of, 112. 

Valleix's points, 68. 

Variola, multiple neuritis following, 388 ; 
myelitis following, 453. 

Vaso-motor changes in tabes, 655. 

Vaso-motor nerves, 397. 

Veins, cerebral, 257 ; spinal, 458. 

Venesection in apoplexy, 238. 

Vermiform process of the cerebellum, 205. 

Vertebral artery, aneurism of, 253. 

Vertigo, 99 ; epileptic, 581 ; laryngeal, 
101 ; Meniere's, 99, 103 ; in multiple 
sclerosis, 620 ; nasal, 76 ; ocular, 48, 
100 ; paralyzing, 101 ; a stomacho laeso, 
101. 

Visual centre, 30. 

Visual field, contraction of, in hysteria, 
541 ; contraction of, in tabes, 631 ; con- 
traction of, in traumatic neuroses, 563 ; 
erroneous projection of, 48 ; examina- 
tion of, 36 ; sectorial defects of, 33. 

Vocal cords, paralysis of, 113 ; spasm of, 
114. 

Voice in paralysis agitans, 503. 

Vomiting, in brain tumor, 296 ; cerebellar, 



IXDEX. 



206 ; cerebral, 12 ; in Graves' disease, ! Word-deafness, 176. 



521 ; hysterical, 5.14. 

Weir Mitchell treatment, 567. 
Westphal's sign in tabes, 650. 
White substance of cord, 439. 
Willis, circle of, 210. 

Vv"or 1-blindness, cf. Psychical blindness, 
170. 



Wrist-drop, 344. 
Writer's cramp, 356. 
Writing, disturbances of, 176. 
Wry-neck, 137. 

Zones, hysterogenic, 551. 
Zoster, herpes, 364, 400. 
Zoster ophthalmicus, 69. 



THE END 



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